At least for me, as a family physician, I usually start with the least invasive and most cost-effective tests, and in the case described, an ischemic problem in the lower limbs is the ankle-brachial index.

A 70-year-old woman, hypertensive and diabetic, who has worked as a sales clerk all her life. She consults for an internal malleolar lesion in the right leg with intense pain throughout the day that worsens at night. On examination, there are uncomplicated varicose cords in both extremities and normal femoral and popliteal pulses with absence of distal pulses. What examination should we perform first?

Does the clinical examination predict lower extremity peripheral arterial disease?

Lower extremity peripheral arterial disease (PAD) is common and associated with significant increases in morbidity and mortality. Physicians typically depend on the clinical examination to identify patients who need further diagnostic testing. To systematically review the accuracy and precision of the clinical examination for PAD. MEDLINE (January 1966 to March 2005) and Cochrane databases were searched for articles on the diagnosis of PAD based on physical examination published in the English language. Included studies compared an element of the history or physical examination with a reference standard of ankle-brachial index, duplex sonography, or angiogram. Seventeen of the 51 potential articles identified met inclusion criteria. Two of the authors independently extracted data, performed quality review, and used consensus to resolve any discrepancies. For asymptomatic patients, the most useful clinical findings to diagnose PAD are the presence of claudication (likelihood ratio [LR], 3.30; 95% confidence interval [CI], 2.30-4.80), femoral bruit (LR, 4.80; 95% CI, 2.40-9.50), or any pulse abnormality (LR, 3.10; 95% CI, 1.40-6.60). While none of the clinical examination features help to lower the likelihood of any degree of PAD, the absence of claudication or the presence of normal pulses decreases the likelihood of moderate to severe disease. When considering patients who are symptomatic with leg complaints, the most useful clinical findings are the presence of cool skin (LR, 5.90; 95% CI, 4.10-8.60), the presence of at least 1 bruit (LR, 5.60; 95% CI, 4.70-6.70), or any palpable pulse abnormality (LR, 4.70; 95% CI, 2.20-9.90). The absence of any bruits (iliac, femoral, or popliteal) (LR, 0.39; 95% CI, 0.34-0.45) or pulse abnormality (LR, 0.38; 95% CI, 0.23-0.64) reduces the likelihood of PAD. Combinations of physical examination findings do not increase the likelihood of PAD beyond that of individual clinical findings. However, when combinations of clinical findings are all normal, the likelihood of disease is lower than when individual symptoms or signs are normal. A PAD scoring system, which includes auscultation of arterial components by handheld Doppler, provides greater diagnostic accuracy. Clinical examination findings must be used in the context of the pretest probability because they are not independently sufficient to include or exclude a diagnosis of PAD with certainty. The PAD screening score using the hand-held Doppler has the greatest diagnostic accuracy.

Assessment of noninvasive lower extremity arterial testing versus pulse exam.

Palpation of pedal pulses was compared to noninvasive testing in 100 patients referred to a vascular laboratory. Subjects were 65 +/- 13 (mean +/- s.d.) years old. The right dorsalis pedis (DP) artery served as the reference artery for comparison of Doppler studies with physical examination of the pulses. Absolute Doppler pressures in the right DP were 129 +/- 50 mm Hg. The right ankle:brachial index (ABI) was 0.86 +/- 0.32. There were significant differences in ABI in those with (0.68 +/- 0.28) vs without (0.95 +/- 0.31) claudication in either extremity (p < 0.001). Rest pain was also associated with lower ABI (P < 0.04). Diabetics, hypertensives, claudicants and those with ischemic rest pain were less likely to have palpable pulses (P < 0.035). With right DP pressure >/= 118 mm Hg, 63 per cent of subjects had a palpable DP pulse, whereas 68 per cent with ABI > 0.82 had a palpable right DP. Of those (n = 35) with a right DP pressure < 118 mm Hg, only 6 per cent (n = 2) had a palpable pulse, whereas 5 per cent (2/40) with ABI </= 0.82 had palpable DP pulses. Based on these findings, it was predicted that a pulse would likely not be palpable in the left DP with a pressure < 118 mm Hg or with AB </= 0.82. The prediction based on systolic ankle pressures was correct, with 66 per cent sensitivity and 91 per cent specificity. The predication that a left DP pulse would not be palpable with AB </= 0.82 was more accurate (78% sensitive, 97% specific). Presence of a palpable DP pulse suggests the presence of a Doppler pressure >/= 188 mm Hg and ABI > 0.82. The range of ankle pressures with palpable right DP pulses was 64-220 mm Hg, whereas the range with nonpalpable DP was 42-300 mm Hg. Given the frequent disparity of pulse exam and ankle pressures, noninvasive Doppler testing may be necessary for many patients to accurately assess the vascular status of the leg.

Ankle brachial index for the diagnosis of lower limb peripheral arterial disease.

Peripheral arterial disease (PAD) of the lower limb is common, with prevalence of both symptomatic and asymptomatic disease estimated at 13% in the over 50 age group. Symptomatic PAD affects about 5% of individuals in Western populations between the ages of 55 and 74 years. The most common initial symptom of PAD is muscle pain on exercise that is relieved by rest and is attributed to reduced lower limb blood flow due to atherosclerotic disease (intermittent claudication). The ankle brachial index (ABI) is widely used by a variety of healthcare professionals, including specialist nurses, physicians, surgeons and podiatrists working in primary and secondary care settings, to assess signs and symptoms of PAD. As the ABI test is non-invasive and inexpensive and is in widespread clinical use, a systematic review of its diagnostic accuracy in people presenting with leg pain suggestive of PAD is highly relevant to routine clinical practice. To estimate the diagnostic accuracy of the ankle brachial index (ABI) - also known as the ankle brachial pressure index (ABPI) - for the diagnosis of peripheral arterial disease in people who experience leg pain on walking that is alleviated by rest. We carried out searches of the following databases in August 2013: MEDLINE (Ovid SP),Embase (Ovid SP), the Cumulative Index to Nursing and Allied Health Literature (CINAHL) (EBSCO), Latin American and Caribbean Health Sciences (LILACS) (Bireme), Database of Abstracts of Reviews of Effects and the Health Technology Assessment Database in The Cochrane Library, the Institute for Scientific Information (ISI) Conference Proceedings Citation Index - Science, the British Library Zetoc Conference search and Medion. We included cross-sectional studies of ABI in which duplex ultrasonography or angiography was used as the reference standard. We also included cross-sectional or diagnostic test accuracy (DTA) cohort studies consisting of both prospective and retrospective studies.Participants were adults presenting with leg pain on walking that was relieved by rest, who were tested in primary care settings or secondary care settings (hospital outpatients only) and who did not have signs or symptoms of critical limb ischaemia (rest pain, ischaemic ulcers or gangrene).The index test was ABI, also called the ankle brachial pressure index (ABPI) or the Ankle Arm Index (AAI), which was performed with a hand-held doppler or oscillometry device to detect ankle vessels. We included data collected via sphygmomanometers (both manual and aneroid) and digital equipment. Two review authors independently replicated data extraction by using a standard form, which included an assessment of study quality, and resolved disagreements by discussion. Two review authors extracted participant-level data when available to populate 2×2 contingency tables (true positives, true negatives, false positives and false negatives).After a pilot phase involving two review authors working independently, we used the methodological quality assessment tool the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2), which incorporated our review question - along with a flow diagram to aid reviewers' understanding of the conduct of the study when necessary and an assessment of risk of bias and applicability judgements. We screened 17,055 records identified through searches of databases. We obtained 746 full-text articles and assessed them for relevance. We scrutinised 49 studies to establish their eligibility for inclusion in the review and excluded 48, primarily because participants were not patients presenting solely with exertional leg pain, investigators used no reference standard or investigators used neither angiography nor duplex ultrasonography as the reference standard. We excluded most studies for more than one reason.Only one study met the eligibility criteria and provided limb-level accuracy data from just 85 participants (158 legs). This prospective study compared the manual doppler method of obtaining an ABI (performed by untrained personnel) with the automated oscillometric method. Limb-level data, as reported by the study, indicated that the accuracy of the ABI in detecting significant arterial disease on angiography is superior when stenosis is present in the femoropopliteal vessels, with sensitivity of 97% (95% confidence interval (CI) 93% to 99%) and specificity of 89% (95% CI 67% to 95%) for oscillometric ABI, and sensitivity of 95% (95% CI 89% to 97%) and specificity of 56% (95% CI 33% to 70%) for doppler ABI. The ABI threshold was not reported. Investigators attributed the lower specificity for doppler to the fact that a tibial or dorsalis pedis pulse could not be detected by doppler in 12 of 27 legs with normal vessels or non-significant lesions. The superiority of the oscillometric (automated) method for obtaining an ABI reading over the manual method with a doppler probe used by inexperienced operators may be a clinically important finding. Evidence about the accuracy of the ankle brachial index for the diagnosis of PAD in people with leg pain on exercise that is alleviated by rest is sparse. The single study included in our review provided only limb-level data from a few participants. Well-designed cross-sectional studies are required to evaluate the accuracy of ABI in patients presenting with early symptoms of peripheral arterial disease in all healthcare settings. Another systematic review of existing studies assessing the use of ABI in alternative patient groups, including asymptomatic, high-risk patients, is required.

Peripheral vascular examination

Special maneuvers Ankle-brachial pressure index (ABPI) assesses peripheral vascular disease. It may however be unreliable in patients with calcified arteries in the calf (often diabetic patients) or those with extensive oedema, in which case toe pressure or Toe-brachial pressure index (TBPI) should be measured to aid in the diagnosis. Venous refill with dependency (should be less than 30 seconds) – the vein should bulge outward within 30 seconds of elevation for one minute. Buerger's test (assessment of arterial sufficiency):With the patient supine, note the colour of the feet soles. They should be pink. Then elevate both legs to 45 degrees for more than 1 minute. Observe the soles. If there is marked pallor (whiteness), ischemia should be suspected. Next check for rubor of dependency. Sit the patient upright and observe the feet. In normal patients, the feet quickly turn pink. If, more slowly, they turn red like a cooked lobster, suspect ischemia.

InternalMed_Harrison

Noninvasive Testing The history and physical examination are often sufficient to establish the diagnosis of PAD. An objective assessment of the presence and severity of disease is obtained by noninvasive techniques. Arterial pressure can be recorded noninvasively in the legs by placement of sphygmomanometric cuffs at the ankles and the use of a Doppler device to auscultate or record blood flow from the dorsalis pedis and posterior tibial arteries. Normally, systolic blood pressure in the legs and arms is similar. Indeed, ankle pressure may be slightly higher than arm pressure due to pulse-wave amplification. In the presence of hemodynamically significant stenoses, the systolic blood pressure in the leg is decreased. Thus, the ratio of the ankle and brachial artery pressures (termed the ankle:brachial index, or ABI) is 1.00–1.40 in normal individuals. ABI values of 0.91–0.99 are considered “borderline,” and those <0.90 are abnormal and diagnostic of PAD. ABIs >1.40 indicate

Diabetic Foot Care -- Technique or Treatment

Clinical evaluation of vascularity and PAD starts with a thorough medical history (e.g., PAD risk factors, claudication, rest pain, history of non-healing wounds). Palpation of dorsalis pedis and posterior tibial arteries are the baseline for diabetic foot exams, but palpation of popliteal and femoral pulses can further assess the level of PAD. A decrease in pedal pulses may warrant evaluation with a Doppler ultrasound or further non-invasive testing for PAD. A Doppler ultrasound of pedal arteries can reveal triphasic flow (normal), biphasic flow (some arterial disease), monophasic flow (PAD with risk for limb ischemia), or absent (severe PAD and high risk for ischemia and limb loss). Non-invasive vascular tests include vascular labs (i.e., ankle-brachial index (ABI), segmental pressures, pulse volume recording, toe pressures, and transcutaneous partial pressure of oxygen [TcPO2]) and treadmill functional testing. The American Diabetes Association (ADA) recommends a screening ABI in patients with diabetes greater than 50 years old, and if normal, a repeat ABI should take place every five years. Screening ABI in patients with diabetes less 50 years old should be a consideration if they also have PAD risk factors (e.g., smoking, hypertension, hyperlipidemia, diabetes greater than ten years). If any indications of PAD are present during these exams, a referral to vascular surgery should follow. [22] [21]

Peripheral Arterial Disease -- Evaluation

On physical exam, patients with PAD may have diminished or absent lower extremity pulses. This finding can be confirmed with the ankle-brachial index (ABI), an objective, bedside measure of lower extremity arterial perfusion. As its name implies, the ABI compares the systolic blood pressure at the ankle to the systolic pressure in the arm. A manual blood pressure cuff should be placed just above the ankle while locating the posterior tibial artery or dorsalis pedis artery with a handheld Doppler probe. While listening to the Doppler signal, the blood pressure cuff is inflated until it obliterates the Doppler signal. As the cuff slowly deflates, the pressure at which the Doppler signal returns is recorded as the systolic ankle pressure. The same steps are repeated for the other pedal artery and the other leg. Likewise, the brachial pressure can be measured with a blood pressure cuff on the upper arm and a Doppler probe positioned over the radial or ulnar artery at the wrist. The ABI is the highest systolic pressure measured at each ankle divided by the higher of the two systolic brachial pressures. A normal ABI ranges from 0.9 to 1.3. PAD is defined as an ABI less than 0.9 and most patients with claudication have an ABI between 0.5 and 0.9. Patients with extremely low ABI’s (less than 0.5) usually have ischemic rest pain or tissue loss. An ABI greater than 1.3 indicates arterial wall stiffening which can occur in patients with diabetes or renal failure. If a cuff pressure of 250mmHg does not obliterate the Doppler signal, the ABI is classified as “non-compressible.” Patients with falsely elevated (greater than 1.3) or non-compressible ABI’s require alternative imaging or physiologic studies to confirm the diagnosis of PAD. [10]

First_Aid_Step2

Femoropopliteal disease: Calf claudication; pulses below the femoral artery are absent. Acute ischemia: Most often caused by embolization from the heart; acute occlusions commonly occur at bifurcations distal to the last palpable pulse. May also be 2° to cholesterol atheroembolism (“blue toe syndrome”). ■Severe chronic ischemia: Lack of blood perfusion leads to muscle atrophy, pallor, cyanosis, hair loss, and gangrene/necrosis. Carefully palpate pulses and auscultate for bruits. Measurement of ankle and brachial systolic BP (ankle-brachial index, or ABI) can provide objective evidence of atherosclerosis (rest pain usually occurs with an ABI < 0.4). A high ABI can indicate calcification of the arteries. Doppler ultrasound helps identify stenosis and occlusion. Doppler ankle systolic pressure readings that are > 90% of brachial readings are normal. Arteriography and digital subtraction angiography are necessary for surgical evaluation.

Leg ulcers and hydroxyurea: report of three cases with essential thrombocythemia.

CASE 1: A 65-year-old woman with essential thrombocythemia (ET) had been taking oral hydroxyurea (HU), 1,000 mg daily, for 7 years. Six months ago, she developed an ulcer on the outer part of her left ankle, which healed spontaneously within 2 months. She presented with a new, tender, shallow ulcer, 2 cm x 2 cm in size, at the same site. Doppler examination revealed thrombosis of the left common femoral vein and a calcified atheroma plaque of the left common femoral artery. The dosage of HU was decreased to 500 mg daily when the platelet counts were found to be within normal levels. The ulcer completely healed within 2 months with occlusive wound dressings, and has not recurred within the follow-up period of 1 year. CASE 2: A 56-year-old women presented with multiple, painful, leg ulcers of 1 year duration. She had been diagnosed as having ET and had been on HU therapy, 1,500 mg/day, for the past 5 years. Interferon-alpha-2b was started 3 months ago, in addition to HU, which was tapered to 1,000 mg daily. She had suffered from hypertension for 20 years treated with nifedipine and enalapril, and had recently been diagnosed with diabetes mellitus which was controlled by diet. Examination revealed three ulcers located on the lateral aspects of both ankles and right distal toe. Arterial and venous Doppler examinations were within normal limits. Histopathology of the ulcer revealed nonspecific changes with a mixed inflammatory cell infiltrate around dermal vessels. The ulcers completely healed within 10 weeks with topical hydrocolloid dressings. After healing, she was lost to follow-up. A year later, it was learned that she had developed a new ulcer at her right heel, 3 months after her last visit (by phone call). This ulcer persisted for 8 months until HU was withdrawn. CASE 3: A 64-year-old woman with ET presented with a painful leg ulcer of 6 months' duration. She had been taking oral HU for 5 years. She had a 20-year history of hypertension treated with lisinopril. Examination revealed a punched-out ulcer of 2 cm x 2 cm over the right lateral malleolus. Doppler examination of the veins revealed insufficiency of the right greater saphenous and femoral veins. Angiography showed multiple stenoses of the right popliteal and femoral arteries. As her platelet count remained high, HU was continued. During the follow-up period of 13 months, the ulcer showed only partial improvement with local wound care.

Antiphospholipid Syndrome and Acute Postpartum Limb Ischemia.

The diagnosis of Antiphospholipid syndrome (APS) implies the identification of antiphospoholipid antibodies and arterial/venous thrombosis or pregnancy loss. During pregnancy, there is an increased risk of thrombotic complications. Present a case of acute lower limb ischemia in a patient with APS during postpartum period Materials/ Methods: review of a clinical case and available literature Results: Patient diagnosed with APS (triple antibody positive and antecedent of 3 previous abortions) underwent cesarean at 29 weeks of gestation. She was medicated with aspirin 00mg/day and enoxaparin 60mg/day and had discharge on the second postoperative day. After 3 days she came to the emergency department with acute limb ischemia. AngioCT revealed thrombosis of the right iliac axis and pulmonary thromboembolism. Endovenous hypocoagulation with unfractionated heparin was immediately started. Due to the high thrombotic risk associated with any type of surgical intervention and improvement of ischemia with hypocoagulation, it was decided to postpone surgical revascularization. At the 10th day of hospitalization angioCT was repeated with maintenance of the iliac thrombosis and clinically the patient had severe claudication and ankle-arm index of 0.26. On the 16th day of hospitalization (after 5 plasmapheresis sessions), she was submitted to trans-femoral thrombectomy, with a good femoral pulse at the end of the procedure. On the 3rd postoperative day we detected pulse loss and angioCT confirmed re-thrombosis of the iliac axis and an oclusion of the popliteal artery. A new attempt at revascularization was made and a new transfemoral thrombectomy was performed, with immediate pulse recovery. We performed intraoperative angiography that confirmed occlusion of the popliteal artery and we proceeded trans-popliteal thrombectomy. Intraoperative control angiography revealed permeability of ilio- -femoro-popliteal axes with some defects of filling of the crural arteries (anterior tibial and peroneal arteries). After the procedure the patient become assymptomatic, with an ankle-arm index of 0.55 (with normal flow in femoral and popliteal artery but monophasyc flow in distal arteries) and had discharged medicated with aspirin, antivitaminik K and corticoid. The beneficial/risk of revascularization surgery should be well considered as well as the timing in which it should be performed. Plasmapheresis is important to minimize the thrombotic risk associated with the surgical procedure. Intra-operative angiography is essencial since arterial thrombosis can occur in several sectors, which can conditionate the success of revascularization procedure.

[Early diagnosis of arteriopathy of the legs using measures adapted to general practitioners: the systolic index and pulse perception].

Atherosclerotic lower-limb arteriopathy is a serious disease. Its prevalence often underestimated when relying only on questioning the patient. A simple early detection method is the systolic index, i.e. the ratio of systolic ankle over brachial pressure. This ratio should normally be greater than 0.9. Lower values indicate detectable arterial obstruction as revealed by reduced peripheral perfusion. Under 0.75 patients are considered as suffering from peripheral vascular disease and require further investigations and specialist opinion. With an index between 0.75 and 0.90 patients are classified as stage I or II of the disease. In a survey by 180 General Practitioners, which were trained by angiologists to measure ankle pressure, more than 1,400 patients, between 40 and 75-years old, with at least one vascular risk factor (hypertension, diabetes, lipids, tobacco) were selected. Tobacco was the most prevalent vascular risk factor, then diabetes (particularly in men). 23.8% of patients recorded values between 0.90 and 0.75 and thus suffering from peripheral vascular disease (certainly age dependent). Clinically absent pulses at the posterior tibial and the dorsalis pedis artery were found is 19 and 27% of patients respectively. Most of the patients received a pharmacological substance allowing to check this index sensitivity. Thus systolic index is a simple low cost method for early detection and care and more general utilisation is proposed.

Popliteal Entrapment Syndrome as a Cause of Chronic Lower Extremity Pain in a 16-Year Old.

Popliteal entrapment syndrome is an uncommon cause of intermittent claudication in young patients lacking atherosclerotic risk factors. ZS is a 16-year-old cisgender female with type 1 diabetes complicated by microalbuminuria, obesity (body mass index (BMI) = 45.86 kg/m²), and a history of perinatal stroke with residual right-sided hemiparesis, who presented with six months of worsening bilateral, exertional lower extremity pain. Common causes of chronic bilateral lower extremity pain include peripheral vascular disease and diabetic neuropathy. Less common etiologies include trauma, infection, or juvenile idiopathic arthritis. Given her risk factors, the patient's pain was initially managed as a diabetic neuropathy with pregabalin. Symptoms failed to improve, and she re-presented with positional coolness of the right lower extremity, diminished pulses of the bilateral lower extremities, and weakness in her toes. CT angiography demonstrated occlusion of the right distal superficial femoral and popliteal arteries and diffused tibial disease. Ultimately, the patient was discovered to have right-sided femoral-popliteal occlusion, and she required urgent femoral-tibial bypass. Despite an initial improvement in symptoms postoperatively, she continued to have lower extremity pain and recurrent arterial thrombi, even with antiplatelet and anticoagulation therapy. Eventually, the patient required a right-sided below the knee amputation. This case highlights the high index of suspicion that clinicians must have in young patients with lower extremity pain, both with and without atherosclerotic risk factors, as early intervention facilitates better outcomes. Introduction.

[Asymptomatic arteriopathy of the lower limbs. Prevalence and risk factors in a population of southern Italy].

Peripheral vascular diseases, both symptomatic and asymptomatic, are strong predictors of total and cardiovascular mortality. The commonly used Rose questionnaire, although highly specific, has a low sensitivity to detecting peripheral vascular disease and is not adequate for asymptomatic subjects. Doppler ultrasound measurement of the ankle/brachial systolic blood pressure ratio is a non-invasive, reproducible and accurate method of assessment of peripheral vascular disease and has been validated by angiography. METHODS. Five hundred and ten subjects, corresponding to fifty percent of the people working as civil servants in the Catanzaro city hall, were invited to join the study by a letter. Three hundred and eighty four participated. Exclusion criterion was claudicatio intermittens as detected by Rose questionnaire. All the subjects filled a questionnaire to assess coronary heart disease risk factors and underwent a full clinical examination. Brachial blood pressure was measured on both arms with participants in supine position, just before ECG. The systolic ankle blood pressure was measured with ultrasonic technique. The blood pressure cuff was placed just proximal to the medial malleolus. The ankle-brachial systolic pressure index (Winsor Index) was determined by dividing the highest of the posterior tibial or dorsalis pedis pressures by the highest brachial pressure. A limit of 0.95 was chosen to identify subjects with peripheral arterial disease. Venous blood for serum cholesterol and triglycerides, apolipoprotein AI and B and blood glucose, was collected after an overnight fasting, into Vacutainer Tubes (Becton & Dickinson). RESULTS. No subject had claudicatio intermittens. Sixteen subjects were excluded from the statistical analysis because of missing data. Two hundred and sixty-three were males and 105 females. Twenty-one (5.7%) out of 368 participants had a Winsor Index < 0.95 in at least one leg. These subjects had higher values of systolic and diastolic blood pressure compared to normal subjects whereas no differences were observed with regard to age, BMI, lipid profile and blood glucose. Furthermore the prevalence of hypertension was higher in the group of subjects with asymptomatic peripheral arterial disease. The prevalence of other risk factors for atherosclerosis (cigarette smoking, hyperlipidemia, diabetes mellitus, obesity) was similar in subjects with or without peripheral arterial disease. DISCUSSION. In the present study the prevalence of Winsor Index < 0.95 was 5.7%, similar to that reported by other authors. Hypertension was the only risk factor for atherosclerosis associated with peripheral arterial disease. Other authors also reported a higher prevalence of cigarette smoking among subjects with peripheral disease. In our population this association was not found but the participants were younger and consequently the exposure to this risk factor was shorter. CONCLUSION. The measurement of systolic ankle blood pressure by Doppler ultrasounds is a non-invasive, well accepted, highly specific and sensitive method to detect asymptomatic peripheral arterial disease. It might be of value in better defining the cardiovascular risk profile both in epidemiologic studies and clinical practice, especially in subjects with hypertension.

Aortoiliac occlusive disease

Diagnosis The physical examination usually shows weakened femoral pulses and a reduced ankle-brachial index. The diagnosis can be verified by color duplex scanning, which reveals either a peak systolic velocity ratio ≥2.5 at the site of stenosis and/or a monophasic waveform. MRA and multidetector CTA are often used to determine the extent and type of obstruction. Another technique is digital subtraction angiography which allows verification of the diagnosis and endovascular treatment in a single session. Angiography provides important information regarding the perfusion and patency of distal arteries (e.g. femoral artery). The presence of collateral arteries in the pelvic and groin area is important in maintaining crucial blood flow and lower limb viability. However, angiography should only be used if symptoms warrant surgical intervention.

Popliteal artery pseudoaneurysm after total knee replacement.

We report the case of a popliteal pseudoaneurysm following total knee replacement. A 70-year-old woman underwent total left knee replacement because of severe osteoarthritis. Eight days later she presented with oedema and pain in her left calf She had palpable foot pulses on the left leg and the ankle-brachial index was 0.98. The patient was treated for deep vein thrombosis. Two days later her calf pain and oedema deteriorated and her distal pulses were no longer palpable, while she developed limb coldness and paraesthesia, and the ankle-brachial index dropped to 0.4. Sonography was urgently performed indicating a large popliteal artery aneurysm (5.8 x 6.9 x 7.2 cm), confirmed by angiography. The patient was managed with removal of a 3.5 cm long segment of the popliteal artery and reconstruction with synthetic graft (PTFE 6 mm). Her condition soon improved and the patient is capable of walking approximately 1 km per day at 18-month follow-up.

Distal pulse palpation: is it reliable?

The aim of this study was to evaluate the reliability of distal pulse palpation. The dorsalis pedis and the tibialis posterior arteries of 25 patients with suspected lower limb arterial disease were independently palpated by three vascular surgeons and three medical students in the outpatient clinic and by two vascular nurses and one physician in the vascular laboratory. The palpation findings were compared to the ankle/brachial index (ABI). Palpable and unpalpable pulses were best separated with ABI 0.76 as the cutoff point. The degree of misdiagnosis was unacceptably high, with an underdiagnosis of more than 30%. The agreement was highest (kappa 0. 68, good) among the vascular laboratory personnel in the peaceful vascular laboratory and lowest (kappa 0.38, fair) among the vascular surgeons in the busy outpatient clinic. The poor agreement and the high proportion of misdiagnosis obtained in the outpatient clinic argue against the use of pulse palpation as a single diagnostic method. Palpable pulses with low ABIs clearly state the need for more objective measurements whenever ischemia is suspected. Yet, by carefully palpating both pedal arteries under good, nonhurried conditions the reproducibility and accuracy of pulse palpation can be tolerable.

Acute compartment syndrome of the foot following fixation of a pilon variant ankle fracture.

Acute traumatic compartment syndrome of the foot is a serious potential complication after fractures, crush injuries, or reperfusion injury after vascular repair. Foot compartment syndrome in association with injuries to the ankle is rare. This article presents a case of acute compartment syndrome of the foot following open reduction and internal fixation of an ankle fracture. A 16-year-old girl presented after sustaining a left ankle injury. Radiographs demonstrated a length-stable posterior and lateral malleolar ankle fracture. Initial treatment consisted of a bulky splint and crutches pending the improvement of her swelling. Over the course of a week, the soft tissue environment of the distal lower extremity improved, and the patient underwent open reduction and internal fixation of both her fibula and distal tibia through 2 approaches. Approximately 2 hours from the completion of surgery, the patient reported worsening pain over the medial aspect of her foot and into her calcaneus. Physical examination of the foot demonstrated a swollen and tense abductor hallicus and heel pad. Posterior tibial and dorsalis pedis pulses were palpable and her sensation was intact throughout her foot. Emergently, fasciotomy of both compartments was performed through a medial incision. Postoperatively, the patient reported immediate pain relief. At 18-month follow-up, she reported no pain and had returned to all of her preinjury athletic activities.

Blunt trauma

Injury to extremities (like arms, legs, hands, feet) is extremely common. Falls are the most common etiology, making up as much as 30% of upper and 60% of lower extremity injuries. The most common mechanism for solely upper extremity injuries is machine operation or tool use. Work related accidents and vehicle crashes are also common causes. The injured extremity is examined for four major functional components which include soft tissues, nerves, vessels, and bones. Vessels are examined for expanding hematoma, bruit, distal pulse exam, and signs/symptoms of ischemia, essentially asking, “Does blood seem to be getting through the injured area in a way that enough is getting to the parts past the injury?” When it is not obvious that the answer is “yes”, an injured extremity index or ankle-brachial index may be used to help guide whether further evaluation with computed tomography arteriography. This uses a special scanner and a substance that makes it easier to examine the vessels in

[Use of the ankle-brachial index in the detection of peripheral arterial disease of the lower extremities in general medicine].

Peripheral arterial disease of the lower extremities (PAD) is a serious condition, frequently under-evaluated. Long asymptomatic, it is easily detected by measuring the ankle-brachial index (ABI), a reference tool that is reliable, reproducible, simple and inexpensive. The objective of this thesis was to determine the rate of achievement of ABI in French Haute Autorité de santé indications, identify the associated factors and prioritize the obstacles to achieving ABI. Descriptive and analytical epidemiological study, with analysis of practices, prospectively addressed by postal questionnaire to a randomized sample of 220 general practitioners practicing in the European Metropolis of Lille between December 15, 2016 and February 15, 2017. Our sample consisted of 92 GPs (42% participation). Among them, only 6 practiced ABI, notably for: intermittent claudication (n=5: 5%, IC95% [1; 10]), the existence of at least 2 cardiovascular risk factors (n=2: 2%, IC95% [0; 5]), diabetic patients over 40 years of age (n=2: 2%, IC95% [0; 5]), patients with diabetes (n=2: 2%, IC95% [0; 5]), patients with diabetes (n=2: 1%, IC95% [0; 5]), patients with diabetes (n=2: 1%, IC95% [0; 5]), and patients with diabetes (n=2: 1%, IC95% [0; 5]): 2%, CI95% [0; 5]), patients over 50 years of age with a history of diabetes or smoking (n=2: 2%, CI95% [0; 5]), or those with an unhealed lower extremity skin lesion (n=5: 5%, CI95% [1; 10]). The most frequently cited barriers were: the prescription of a routine echo-doppler (61%, 95% CI [51; 71]), lack of control (46%, 95% CI [36; 56]), time considered too long (17%, 95% CI [10; 25]), and equipment purchase or maintenance (19%, 95% CI [10.5; 26.4]). ABI is few used in our sample, mainly due to delegation to angiologists.

First_Aid_Step2

Hospitalized patients should receive DVT prophylaxis consisting of exercise as tolerated, anti-thromboembolic stockings, and SQ unfractionated heparin or low-molecular-weight heparin. Occlusion of the blood supply to the extremities by atherosclerotic plaque. The lower extremities are most commonly affected. Clinical manifestations depend on the vessels involved, the extent and rate of obstruction, and the presence of collateral blood ﬂ ow. Presents with intermittent claudication (reproducible leg pain that occurs with walking and is always relieved with rest). As the disease progresses, pain occurs at rest and affects the distal extremities. Dorsal foot ulcerations may develop 2° to poor perfusion. A painful, cold, numb foot is characteristic of critical limb ischemia. Aortoiliac disease: Associated with Leriche’s syndrome (buttock claudication, ↓ femoral pulses, male impotence). Femoropopliteal disease: Calf claudication; pulses below the femoral artery are absent.

Spontaneous auto-amputation of the foot in a case of diabetes, atherosclerosis and gangrene.

Auto-amputation is the spontaneous separation of non-viable tissue from viable tissue, and is usually associated with dry gangrene, occurring in the distal portions of the lower extremities. A 68-year-old female presented with dry gangrene of both feet and subsequent loss of the right foot and digits on the left foot over a period of one and half years. After about six months disarticulation of the right foot from the ankle joint resulted. She gradually lost the digits of the left foot two months later. She had pallor and showed absence of popliteal and distal pulses on both sides. The left foot showed absence of the digits with exposed 3rd and 4th metatarsals and some areas of gangrene and sloughing. Her Hb was 5.4 g/dl and random blood sugar was 340 mg%. Doppler study of the lower limbs showed blockage at the level of the superficial femoral artery on the right side and a patent popliteal artery on the left. She was managed conservatively as she was unwilling for surgical intervention.

High prevalence of exercise-induced ischemia in the asymptomatic limb of patients with apparently strictly unilateral symptoms and unilateral peripheral artery disease.

The prevalence of exercise-induced ischemia in the asymptomatic limb of patients with unilateral claudication based on history and treadmill evaluation, and with unilateral ipsilateral peripheral artery disease (i.e ankle-to-brachial systolic pressure index <0.90) is unknown. We detected exercise-induced ischemia in the asymptomatic limb of patients with apparently unilateral claudication. Among 6059 exercise-oximetry tests performed in 3407 nondiabetic and 961 diabetic patients. We estimated the intensity of ischemia in the both limb (buttocks and calves) using the lowest minimum value of the decrease from rest of oxygen pressure (DROP; limb changes minus chest changes from rest), with significant ischemia defined as DROP lower than -15 mmHg. We found 152 tests performed in 142 nondiabetic patients and 40 tests performed in 38 diabetic patients. The asymptomatic limb showed significant ischemia in 46.7% and 37.5% of the tests. Strictly unilateral exercise-induced claudication with apparently unilateral peripheral artery disease was rare (<4% of all tests). However, among these highly selected tests, significant ischemia was found in the asymptomatic limb in more than one-third of cases. The asymptomatic limb of patients with peripheral artery disease should not be considered a normal limb.

Stenting for peripheral artery disease of the lower extremities: an evidence-based analysis.

In January 2010, the Medical Advisory Secretariat received an application from University Health Network to provide an evidentiary platform on stenting as a treatment management for peripheral artery disease. The purpose of this health technology assessment is to examine the effectiveness of primary stenting as a treatment management for peripheral artery disease of the lower extremities. CONDITION AND TARGET POPULATION Peripheral artery disease (PAD) is a progressive disease occurring as a result of plaque accumulation (atherosclerosis) in the arterial system that carries blood to the extremities (arms and legs) as well as vital organs. The vessels that are most affected by PAD are the arteries of the lower extremities, the aorta, the visceral arterial branches, the carotid arteries and the arteries of the upper limbs. In the lower extremities, PAD affects three major arterial segments i) aortic-iliac, ii) femoro-popliteal (FP) and iii) infra-popliteal (primarily tibial) arteries. The disease is commonly classified clinically as asymptomatic claudication, rest pain and critical ischemia. Although the prevalence of PAD in Canada is not known, it is estimated that 800,000 Canadians have PAD. The 2007 Trans Atlantic Intersociety Consensus (TASC) II Working Group for the Management of Peripheral Disease estimated that the prevalence of PAD in Europe and North America to be 27 million, of whom 88,000 are hospitalizations involving lower extremities. A higher prevalence of PAD among elderly individuals has been reported to range from 12% to 29%. The National Health and Nutrition Examination Survey (NHANES) estimated that the prevalence of PAD is 14.5% among individuals 70 years of age and over. Modifiable and non-modifiable risk factors associated with PAD include advanced age, male gender, family history, smoking, diabetes, hypertension and hyperlipidemia. PAD is a strong predictor of myocardial infarction (MI), stroke and cardiovascular death. Annually, approximately 10% of ischemic cardiovascular and cerebrovascular events can be attributed to the progression of PAD. Compared with patients without PAD, the 10-year risk of all-cause mortality is 3-fold higher in patients with PAD with 4-5 times greater risk of dying from cardiovascular event. The risk of coronary heart disease is 6 times greater and increases 15-fold in patients with advanced or severe PAD. Among subjects with diabetes, the risk of PAD is often severe and associated with extensive arterial calcification. In these patients the risk of PAD increases two to four fold. The results of the Canadian public survey of knowledge of PAD demonstrated that Canadians are unaware of the morbidity and mortality associated with PAD. Despite its prevalence and cardiovascular risk implications, only 25% of PAD patients are undergoing treatment. The diagnosis of PAD is difficult as most patients remain asymptomatic for many years. Symptoms do not present until there is at least 50% narrowing of an artery. In the general population, only 10% of persons with PAD have classic symptoms of claudication, 40% do not complain of leg pain, while the remaining 50% have a variety of leg symptoms different from classic claudication. The severity of symptoms depends on the degree of stenosis. The need to intervene is more urgent in patients with limb threatening ischemia as manifested by night pain, rest pain, ischemic ulcers or gangrene. Without successful revascularization those with critical ischemia have a limb loss (amputation) rate of 80-90% in one year. Diagnosis of PAD is generally non-invasive and can be performed in the physician offices or on an outpatient basis in a hospital. Most common diagnostic procedure include: 1) Ankle Brachial Index (ABI), a ratio of the blood pressure readings between the highest ankle pressure and the highest brachial (arm) pressure; and 2) Doppler ultrasonography, a diagnostic imaging procedure that uses a combination of ultrasound and wave form recordings to evaluate arterial flow in blood vessels. The value of the ABI can provide an assessment of the severity of the disease. Other non invasive imaging techniques include: Computed Tomography (CT) and Magnetic Resonance Angiography (MRA). Definitive diagnosis of PAD can be made by an invasive catheter based angiography procedure which shows the roadmap of the arteries, depicting the exact location and length of the stenosis / occlusion. Angiography is the standard method against which all other imaging procedures are compared for accuracy. More than 70% of the patients diagnosed with PAD remain stable or improve with conservative management of pharmacologic agents and life style modifications. Significant PAD symptoms are well known to negatively influence an individual quality of life. For those who do not improve, revascularization methods either invasive or non-invasive can be used to restore peripheral circulation.  TECHNOLOGY UNDER REVIEW: A Stent is a wire mesh "scaffold" that is permanently implanted in the artery to keep the artery open and can be combined with angioplasty to treat PAD. There are two types of stents: i) balloon-expandable and ii) self expandable stents and are available in varying length. The former uses an angioplasty balloon to expand and set the stent within the arterial segment. Recently, drug-eluting stents have been developed and these types of stents release small amounts of medication intended to reduce neointimal hyperplasia, which can cause re-stenosis at the stent site. Endovascular stenting avoids the problem of early elastic recoil, residual stenosis and flow limiting dissection after balloon angioplasty. In individuals with PAD of the lower extremities (superficial femoral artery, infra-popliteal, crural and iliac artery stenosis or occlusion), is primary stenting more effective than percutaneous transluminal angioplasty (PTA) in improving patency?In individuals with PAD of the lower extremities (superficial femoral artery, infra-popliteal, crural and iliac artery stenosis or occlusion), does primary stenting provide immediate success compared to PTA?In individuals with PAD of the lower extremities (superficial femoral artery, infra-popliteal, crural and iliac artery stenosis or occlusion), is primary stenting associated with less complications compared to PTA?In individuals with PAD of the lower extremities (superficial femoral artery, infra-popliteal, crural and iliac artery stenosis or occlusion), does primary stenting compared to PTA reduce the rate of re-intervention?In individuals with PAD of the lower extremities (superficial femoral artery, infra-popliteal, crural and iliac artery stenosis or occlusion) is primary stenting more effective than PTA in improving clinical and hemodynamic success?Are drug eluting stents more effective than bare stents in improving patency, reducing rates of re-interventions or complications?  A literature search was performed on February 2, 2010 using OVID MEDLINE, MEDLINE In-Process and Other Non-Indexed Citations, OVID EMBASE, the Cochrane Library, and the International Agency for Health Technology Assessment (INAHTA). Abstracts were reviewed by a single reviewer and, for those studies meeting the eligibility criteria, full-text articles were obtained. Reference lists were also examined for any additional relevant studies not identified through the search. The quality of evidence was assessed as high, moderate, low or very low according to GRADE methodology. English language full-reports from 1950 to January Week 3, 2010Comparative randomized controlled trials (RCTs), systematic reviews and meta-analyses of RCTsProven diagnosis of PAD of the lower extremities in all patients.Adult patients at least 18 years of age.Stent as at least one treatment arm.Patency, re-stenosis, re-intervention, technical success, hemodynamic (ABI) and clinical improvement and complications as at least an outcome. Non-randomized studiesObservational studies (cohort or retrospective studies) and case reportFeasibility studiesStudies that have evaluated stent but not as a primary intervention The primary outcome measure was patency. Secondary measures included technical success, re-intervention, complications, hemodynamic (ankle brachial pressure index, treadmill walking distance) and clinical success or improvement according to Rutherford scale. It was anticipated, a priori, that there would be substantial differences among trials regarding the method of examination and definitions of patency or re-stenosis. Where studies reported only re-stenosis rates, patency rates were calculated as 1 minus re-stenosis rates. Odds ratios (for binary outcomes) or mean difference (for continuous outcomes) with 95% confidence intervals (CI) were calculated for each endpoint. An intention to treat principle (ITT) was used, with the total number of patients randomized to each study arm as the denominator for each proportion. Sensitivity analysis was performed using per protocol approach. A pooled odds ratio (POR) or mean difference for each endpoint was then calculated for all trials reporting that endpoint using a fixed effects model. PORs were calculated for comparisons of primary stenting versus PTA or other alternative procedures. Level of significance was set at alpha=0.05. Homogeneity was assessed using the chi-square test, I(2) and by visual inspection of forest plots. If heterogeneity was encountered within groups (P < 0.10), a random effects model was used. All statistical analyses were performed using RevMan 5. Where sufficient data were available, these analyses were repeated within subgroups of patients defined by time of outcome assessment to evaluate sustainability of treatment benefit. (ABSTRACT TRUNCATED)

Peripheral artery disease

High risk populations Peripheral arterial disease is more common in these populations: All people who have leg symptoms with exertion (suggestive of claudication) or ischemic rest pain All people aged 65 years and over regardless of risk factor status All people between 50 and 69 and who have a cardiovascular risk factor (particularly diabetes or smoking) Age less than 50 years, with diabetes and one other atherosclerosis risk factor (smoking, dyslipidemia, hypertension, or hyperhomocysteinemia) Individuals with an abnormal lower extremity pulse examination Those with known atherosclerotic coronary, carotid, or renal artery disease All people with a Framingham risk score of 10%–20% All people who have previously experienced chest pain Mechanism

[Radiation arteritis- and radiodermitis-induced leg ulcer: surgical revascularization].

A forty-four-year old man was hospitalized for diagnosis and treatment of a left leg ulcer which did not heal despite good compliance with a three-month medical regimen. Twenty years before he had undergone surgical curettage and radiotherapy (81 gy) for an osteosarcoma of the upper third of the left tibia. He was considered completely cured with regular findings. On examination he had a 5 X 7 cm deep ulcer with raised margins and no signs of infection, localized on the radiodermatitis on the medial aspect of his left leg. Arterial examination confirmed the left arteriopathy with absence of distal pulses; the Ankle Brachial Pressure Index was 0.69 and the foot TcPO2 27 mmHg. Arteriography confirmed the localized left lesions with three distal popliteal and proximal arterial occlusions, all other arteries being strictly normal. Arterial and dermatological radiation leg ulcer was retained as the etiological diagnosis. As the ulcer was very painful, extensive and limited walking distance, surgical revascularisation was undertaken because endoluminal revascularization was impossible. A femoroperoneal saphenous bypass was performed with surgical incisions beyond the radiodermatitis area. Two months after a split skin graft, the ulcer was considered healed and the patency of the by-pass confirmed on duplex examination. This is the first case report of a successful distal by-pass performed for radiation arteritis and ulcer healing. Long-term follow up should be reported.

Variability of ankle-arm index in general population according to its mode of calculation.

Ankle-arm index (AAI) is commonly used in epidemiological studies on peripheral arterial disease but its mode of calculation varies throughout the literature. We aimed to study the variance of the different measurements required to calculate the AAI according to different formulas, in order to find out the best way of AAI calculation, based on its lesser variability. A sample of 222 subjects from the general population was prospectively studied. Ten persons were excluded because of the presence of intermittent claudication or due to a lack of a Doppler posterior tibial artery (PT) signal. The systolic pressures of both arms, the dorsalis pedis arteries (DP) and the PT were finally measured in 212 healthy persons (mean age 49.9, range 18-101). Methods using the lowest ankle artery pressure were excluded, as an absent pedal pulse may not be abnormal. The mode using the lowest variance was the choice of the highest pressure between PT and DP of each leg divided by the mean of the systolic pressures of both arms. We recommend this mode of AAI calculation in epidemiological studies. As the variances of the measurements used are the lowest, it would be easier thereafter to determine normal ranges and cutoff points.

Cardiovascular examination

From head to toe, there are several places in the body where arterial pulses can be felt for their strength and quality. Many arteries that are on both sides of the body (bilateral) may be palpated at the same time for the sake of comparison, except for the carotid arteries; deep palpation of both carotid arteries can cut off blood flow to the brain. The carotid arteries, should be auscultated to listen for bruits, as this can indicate the buildup of plaque inside of the vessel (also known as atherosclerosis). The brachial arteries may be palpated underneath the biceps tendon. The radial pulses can be found on the forearm, above the wrists. Femoral pulses can be palpated below the inguinal ligament, about halfway between the pubic symphysis and the anterior superior iliac spine. The popliteal arteries are harder to palpate, but can be found by pressing deeply with the knee slightly bent. If the femoral pulse is strong, but the popliteal pulse is weak, this can indicate

Tourniquets revisited.

Controversy swirls about optimal control of life-threatening hemorrhage from an injured extremity whether in combat in the Middle East or in trauma care at home. Left unanswered are four critical questions: (1) What is the simplest tourniquet available? (2) Can it be used below the elbow and the knee? (3) Is pain a factor? (4) What data support so called "Pressure Points?" To address these questions, we measured the effects of three common tourniquets on arterial pulses (Doppler signals) at wrist and ankle of 10 healthy adult volunteers of either sex. We recorded ease of application (1-3, with 3 easiest) by the applicant and pain experienced by the subject (none, light, moderate, severe). Tourniquets were applied sequentially to arm, forearm, thigh, and leg. Tourniquet success was defined as sustained elimination of distal pulse. Pressure points were brachial artery in arm and cubital fossa, common femoral artery (groin), and popliteal artery (knee). The same criteria defined success. All numerical data were meaned and standard error (SE) computed. Significance of apparent differences was assessed with Student's t test for paired observations. Mean age was 36.5 +/- 6.0 years; blood pressure was 123 +/- 6/72 +/- 4 mm Hg. All three tourniquets (sphygmomanometer, 1/2 inch rubber tubing, cloth and windlass) were successful in all patients in all four locations with two exceptions. Thighs of two subjects were too large for the sphygmomanometer and one person experienced test terminating pain with the rubber tube on arm and thigh and with the cloth and windlass on the thigh. Manual (digital) occlusion of the brachial artery in the arm was possible in all but one subject; however, the Doppler signal at the wrist returned within 40.6 +/-6.5 seconds in all but one of the other nine subjects. Pressure point control of the common femoral artery resulted in identical findings except that the pulse returned within 20.6 +/- 4.7 seconds despite sustained pressure. Attempts at control of the brachial artery at the elbow and the popliteal artery at the knee were less successful. Our data indicate that all tourniquets can be used successfully below the knee or elbow. The cloth and windlass is the easiest to apply. It is probably the most readily available or simplest to procure/improvise. Pain is irrelevant. "Pressure Point Control" of extremity arterial hemorrhage is a euphemistic misnomer.

Exertional leg symptoms other than intermittent claudication are common in peripheral arterial disease.

Epidemiological data show that most community-dwelling men and women with lower-extremity peripheral arterial disease (PAD) do not have typical symptoms of intermittent claudication. We compared the prevalence of intermittent claudication, leg symptoms other than intermittent claudication, and absence of exertional leg symptoms between patients with PAD identified from a blood flow laboratory (group 1), patients with PAD in a general medicine practice (group 2), and control patients without PAD (group 3). Numbers of participants in groups 1, 2, and 3 were 137, 26, and 105, respectively. Patients with previously diagnosed PAD were excluded from groups 2 and 3. All participants underwent ankle-brachial index measurement and were administered the San Diego claudication questionnaire to assess leg symptoms. Within groups 1, 2, and 3, prevalences of intermittent claudication were 28.5% (n = 39), 3.8% (n = 1), and 3.8% (n= 4), respectively. Prevalences of exertional leg symptoms other than intermittent claudication were 56.2% (n= 77), 42.3% (n= 11), and 19.0% (n = 20), respectively. Absence of exertional leg symptoms was reported by 15.3% (n= 21), 53.8% (n= 14), and 77.1% (n=81), respectively. Among patients with PAD, older age, male sex, diabetes mellitus, and group 2 vs group 1 status were associated independently with absence of exertional leg symptoms in multivariable regression analysis. Lower ankle-brachial index levels and group 1 vs group 2 status were associated with intermittent claudication. Clinical manifestations of PAD are diverse, particularly among patients identified by ankle-brachial index screening. Exertional leg symptoms other than intermittent claudication are common in PAD. Patients with PAD who are older, male, diabetic, or identified with ankle-brachial index screening in a primary care setting are more likely to have asymptomatic PAD.

Peripheral vascular examination

Arterial pulses Dorsalis pedis artery pulse – on dorsal surface of the foot, running lateral to the tendon of the first toe Posterior tibial artery pulse – posterior and inferior to the medial malleolus Popliteal artery pulse – behind the knee, typically done with both hands Femoral artery pulse – in the femoral triangle / halfway between the ASIS and pubic tubercle Auscultation For femoral artery bruits

Persistent sciatic artery aneurysm: a case report.

In early embryologic development the sciatic artery provides the blood supply to the lower limb bud. When the femoral artery develops, the sciatic artery involutes. However, in rare cases, the sciatic artery persists. It can be visualised as a prolongation of the inferior gluteal artery (branch of the internal iliac artery) and it accompanies the sciatic nerve at the posterior side of the hip. We present the case of a 47-year old woman who consulted because of a numb right foot which presented colder and paler than the left foot. She also had a right painful calf, especially after exercise. Upon physical examination and doppler ultrasound there were no popliteal nor pedal artery pulses in the right leg. CT angiography showed bilateral persistent sciatic arteries with aneurysm formation and the presence of an embolus in the popliteal artery at the right side. Treatment involved thrombolysis of the popliteal occlusion, followed by intra-aneurysmatic stent placement and embolectomy of the popliteal artery and its distal branches. Postoperative clinical results were remarkably good and the patients symptoms dissolved. A persistent sciatic artery is a rare vascular anomaly. It is more prone to vasculopathies such as aneurysm formation. In cases of acute ischemia, correct diagnosis and treatment of this anomaly can avoid serious medical consequences.

Get the LEAD out: noninvasive assessment for lower extremity arterial disease using ankle brachial index and toe brachial index measurements.

Lower extremity arterial disease affects approximately one third of individuals 66 years of age and older and has a high risk for nonhealing wounds, infection, and limb loss. Much wound care is given by or under the direction of nurses. Therefore, the assessment and management of these patients presents many opportunities and challenges. Assessment is the cornerstone of effective care, but traditional methods of lower extremity arterial assessment, such as pulse palpation and pain history, are insufficient to determine the presence and extent of ischemia. Recently published national guidelines for assessment and management of patients with lower extremity wounds have recommended using noninvasive tests such as the ankle brachial index and toe brachial index to rule out lower extremity arterial disease, which complicates wound healing. However, the ankle brachial index can be falsely elevated in patients with diabetes and renal failure because of calcification of the arteries, which causes them to be incompressible. In these situations, it has been advised to obtain a toe pressure or toe brachial index because digital arteries are usually less affected by calcification. There is a paucity of data about the knowledge of principles and performance of the ankle brachial index/toe brachial index by nurses, particularly in the United States, using pocket-sized portable Doppler equipment. Therefore, the purpose of this article is to provide an overview and synthesis of relevant studies and published expert opinion regarding noninvasive arterial assessment using ankle brachial and toe brachial indexes as a basis for developing protocols for performing the tests and identifying gaps in research where further investigation is needed.


The fact of being a photoinduced picture, the histology and the age make the diagnosis of PCT more probable (3). The rest of the answers do not fit with the clinical picture or the histology.

The fact of being a photoinduced picture, the histology and the age make the diagnosis of PCT more probable [HIDDEN]. The rest of the answers do not fit with the clinical picture or the histology.

A 60-year-old male patient who reported 10 days ago the appearance of blistering lesions on the dorsum of the hands after sun exposure. The picture is accompanied by skin fragility. Histopathologically there is a subepidermal blister with PAS+ deposits in and around the superficial dermal vessels. The most plausible diagnosis is:

DERMATOLOGY, VENEREOLOGY AND PLASTIC SURGERY

[The morphology of skin damage in porphyria cutanea tarda (PCT)].

Skin biopsies (fresh and old blisters) of the back of the hand and lower arm, respectively, obtained from 24 patients with clinically and biochemically established porphyria cutanea tarda have been examined light and electron microscopically, 15 out of them also by immunofluorescence microscopy (occurrence of IgG, IgM and C3). Light microscopically there were subepidermal blisterings and typical corial broadenings of the capillary wall with deposits of diastase-resistant PAS-positive material in and around the walls of the vessels. Electron microscopically, replications of the capillary basement membrane with deposits of amorphous material were observed. IgG and C 3 on the dermoepidermal junction and in the capillaries could be identified by means of the direct immunofluorescence method in a number of cases. The pathogenesis of the lesions of the vessel walls and that of the subepidermal blistering, hitherto interpreted as their consequence, is not yet clarified sufficiently.

Reflectance confocal microscopy in the diagnosis of vesicobullous disorders: case series with pathologic and cytologic correlation and literature review.

Vesicobullous disorders are characterized by intraepidermal or subepidermal blistering resulting from different pathogenetic mechanisms. The diagnosis is generally based on clinical examination and semi-invasive/invasive procedures such as cytology and histopathology. In vivo reflectance confocal microscopy (RCM) is a non-invasive technique for real-time, en face imaging of the epidermis and upper dermis with high resolution close to conventional histopathology. To evaluate RCM features of different vesicobullous diseases and correlate with cytologic and histopathologic examination. Ten patients (6M/4F, age range: 9-81 years) affected by blistering diseases, such as herpes simplex, herpes zoster, Kaposi's varicelliform eruption, pemphigus vulgaris, Hailey-Hailey disease, bullous pemphigoid, and porphyria cutanea tarda were evaluated using a handheld RCM device. In our study, a clear correlation between RCM and Tzanck's test and/or histopathology was observed. RCM allowed in all cases an easy identification of the blister spaces and of the split levels, and in some cases specific features were detected, such as giant keratinocytes in herpes infections and acantholytic cells in pemphigus vulgaris and Hailey-Hailey disease. Reflectance confocal microscopy may support the clinical diagnosis of vesicobullous disorders and indicate to the physician the appropriate patient management and/or the need for further investigation.

Mixed connective tissue disease. A clinical, histologic, and immunofluorescence study of eight cases.

A study of the cutaneous eruptions of eight patients with mixed connective tissue disease (MCTD) was performed to better characterize its dermatopathology and to explore a role for the membrane attack complex of complement C5b-9 in lesional pathogenesis. Nine lesional skin biopsies were obtained from eight patients with MCTD and analyzed by conventional light microscopy. Direct immunofluorescence (IF) and indirect IF using a monoclonal antibody to C5b-9 were applied in six and five cases respectively. The biopsied cutaneous eruptions were characterized clinically as photo-distributed erythematosus annular and/or papulosquamous lesions mimnicking subacute cutaneous lupus erythematosus (SCLE) in five of eight patients as an ill-defined, telangiectatic, scaly patch on the face in one patient, palpable purpura in one patient, and dorsal hand blisters resembling porphyria cutanea tarda (PCT) in another. With the exception of the latter two patients, the histology appeared similar, comprising a cell poor and/or lichenoid interface dermatitis with suprabasilar exocytosis around necrotic keratinocytes in the absence of deep periadnexal or perivascular extension or conspicuous follicular plugging, a pattern similar to that of SCLE. However, the lesions differed from SCLE by virtue of vasculopathic alterations comprising vascular ectasia, hypovascularity, and/or luminal thrombosis confined to the superficial vascular plexus and a sclerodermoid tissue reaction, the latter seen in two cases. One biopsy showed a pustular leukocytoclastic vasculitis (LCV). In another case, a biopsied hand blister demonstrated a PCT-like appearance histologically, namely, pauci-inflammatory subepithelial blister formation with hyalinization of dermal papillae capillaries accompanied by an LCV. There was nuclear keratinocyte decoration with IgG and C5b-9 in all cases studied, accompanied by a positive lupus band test in two cases and homogenous deposition of immunoreactants along the dermoepidermal junction and within vessels in the PCT-like eruption. Granular vascular decoration with immunoreactants including C5b-9 was seen in two LCV cases and in two biopsies from rashes clinically mimicking SCLE. Although the epidermal pathology of MCTD mimicks that of SCLE, a concomitant vasculopathy paralleling that seen in skin lesions of dermatomyositis distinquishes the dermatopathology of MCTD from that of SCLE. Corroborating the role of microangiopathy in the pathogenesis of the skin lesions of MCTD was the demonstration of C5b-9 in blood vessels. The deposition of C5b-9 in keratinocytes may explain the pattern Of IgG decoration of keratinocytes; the formation of plasmalemmal pores may permit binding of immunoglobulin to antigens in the nucleus and/or cytosol. The C 5b-9 complex may be the effector mechanism of epithelial and/or endothelial cell injury in MCTD or may serve to augment the effects of antibody-dependent cellular cytotoxicity.

Porphyria-like cutaneous changes induced by tetracycline hydrochloride photosensitization.

Five patients manifested cutaneous changes indistinguishable from those noted in some porphyric disorders, consisting of fragility, denudation, and blister formation of sun-exposed skin. Microscopical examination showed subepidermal bulla formation and the desposition of PAS-positive, diastase-resistant material and IgG in or around the upper dermal blood vessel walls. There was also electron microscopical evidence of vascular basal lamina reduplication and the deposition of a fine fibrillar material in and around these vessels. However, no abnormal porphyrin formation was noted. All five patients had been receiving 250 mg of tetracycline hydrochloride twice a day for at least six months and had had extensive sun exposure prior to the onset of the condition. For four patients, discontinuing the medication led to complete remission, despite subsequent sun exposure; the fifth patient was much improved, but her skin was still somewhat fragile seven months later. We concluded that these cutaneous changes resulted from a low-grade photosensitization by tetracycline hydrochloride.

Nikolsky's sign

Nikolsky's sign is almost always present in Stevens–Johnson syndrome/toxic epidermal necrolysis and staphylococcal scalded skin syndrome, caused by the exfoliative toxin of Staphylococcus aureus. It is also associated with pemphigus vulgaris and pemphigus foliaceus. It is useful in differentiating between the diagnosis of pemphigus vulgaris or mucous membrane pemphigoid (where the sign is present) and bullous pemphigoid (where it is absent). The Nikolsky sign is dislodgement of intact superficial epidermis by a shearing force, indicating a plane of cleavage in the skin epidermal-epidermal junctions (e.g., desmosomes). The histological picture involves thinner, weaker attachments of the skin lesion itself to the normal skin – resulting in easier dislodgement.

Pemphigus vulgaris

Pemphigus vulgaris is a rare chronic blistering skin disease and the most common form of pemphigus. Pemphigus was derived from the Greek word pemphix, meaning blister. It is classified as a type II hypersensitivity reaction in which antibodies are formed against desmosomes, components of the skin that function to keep certain layers of skin bound to each other. As desmosomes are attacked, the layers of skin separate and the clinical picture resembles a blister. These blisters are due to acantholysis, or breaking apart of intercellular connections through an autoantibody-mediated response. Over time the condition inevitably progresses without treatment: lesions increase in size and distribution throughout the body, behaving physiologically like a severe burn.

Bullosis Diabeticorum: A Rare Presentation with Immunoglobulin G (IgG) Deposition Related Vasculopathy. Case Report and Focused Review.

BACKGROUND Bullosis diabeticorum (BD) is a condition characterized by recurrent, spontaneous, and non-inflammatory blistering in patients with poorly controlled diabetes mellitus. While etiopathogenesis remains unclear, roles of neuropathy, vasculopathy and UV light are hypothesized. Most literature reports negative direct and indirect immunofluorescence findings in diabetics with bullous eruptions. Porphyria cutanea tarda, bullous pemphigoid, epidermolysis bullosa, and pseudoporphyria are other differential diagnoses of bullous lesions, and they must be excluded. CASE REPORT We present a 42-year-old African American male with long standing poorly controlled insulin dependent diabetes mellitus with blisters on his left hand and feet. The blisters were noticed three weeks prior to presentation and, thereafter, rapidly increased in size and spontaneously ruptured. Physical examination revealed a multitude of both roofed and unroofed bullous painless skin lesions. Hematoxylin and eosin (H&E) staining dramatized the dermal-epidermal blistering and re-epithelization process. Direct Immunofluorescence (DIF) was positive for 2 + IgG deposition in the already thickened basement membrane of the capillaries of the superficial vascular plexus. After debridement, his wounds greatly improved with over three months of aggressive wound care. CONCLUSIONS Primary immunologic abnormality likely plays no role in the onset of BD. To date, only one article has reported nonspecific capillary-associated immunoglobulin M and C3. This is the first case of BD with IgG deposition in the superficial capillary basement membrane. Positive findings on DIF suggest vasculopathy. Dermal microangiopathy, secondary to immunologic abnormality, is a possible underlying pathogenesis to bullae formation. Punch biopsy with DIF can be an additional diagnostic modality in the management of such cases.

Pemphigus

and eye doctors, as lesions can affect the eyes and mucous membranes of the oral cavity. Intraorally, it resembles the more common diseases lichen planus and mucous membrane pemphigoid. Definitive diagnosis requires examination of a skin or mucous membrane biopsy by a dermatopathologist or oral pathologist. The skin biopsy is taken from the edge of a blister, prepared for histopathology and examined with a microscope. The pathologist looks for an intraepidermal vesicle caused by the breaking apart of epidermal cells (acantholysis). Thus, the superficial (upper) portion of the epidermis sloughs off, leaving the bottom layer of cells on the "floor" of the blister. This bottom layer of cells is said to have a "tombstone" appearance.

Pseudoporphyria and sunbeds.

Five cases of pseudoporphyria with recurrent blistering and skin fragility especially of the hands are described. Porphyrin studies were normal. The histopathological investigations in three of these cases showed subepidermal bullae consistent with porphyria cutanea tarda. All patients had considerable sunbed exposure before symptoms appeared. One patient was taking naproxen, a weak photosensitizer.

Blistering disorders: diagnosis and treatment.

Blistering diseases are a heterogeneous group of disorders that can affect either skin and mucous membrane, or both, varying in presentation, clinical course, pathohistology, immunopathology and treatment. Not infrequently the diagnosis is delayed. This can result in severe, and sometimes fatal consequences. Although these diseases are rare, it is very important to make an accurate diagnosis based on a combination of clinical profile and laboratory observations. A brief review is presented of the following bullous diseases: pemphigus, paraneoplastic pemphigus, bullous pemphigoid, cicatricial pemphigoid, epidermolysis bullosa acquisita, dermatitis herpetiformis, linear IgA bullous disease, porphyria cutanea tarda, and subcorneal pustular dermatitis. Their clinical, pathohistologic and immunopathologic features and recommendations for therapy are discussed.

Pathology_Robbins

Fig. 24.8 Levelsofblisterformation.(A)Subcorneal(asinpemphigusfoliaceus).(B)Suprabasal(asinpemphigusvulgaris).(C)Subepidermal(asinbullouspemphigoidordermatitisherpetiformis). mechanisms, but differ in their clinical presentation and course due to variation in the location of the target antigen (hemidesmosomes in bullous pemphigoid, desmosomes in pemphigus). Fig.24.12B ).Theoverlyingepidermischaracteristicallylacksacantholysis.Earlylesionsshowvariablenumbersofeosinophilsatthedermal-epidermaljunction,occasionalneutrophils,superficialdermaledema,andassociatedbasalcelllayervacuolization.Thevacuolatedbasalcelllayereventuallygivesrisetoafluid-filledblister( Fig.24.12C ).Theblisterroofconsistsoffull-thicknessepidermiswithintactintercellularjunctions,akeydistinctionfromtheblistersseeninpemphigus.

Pemphigus vulgaris

Pemphigus is an autoimmune disease caused by antibodies directed against both desmoglein 1 and desmoglein 3 present in desmosomes. Loss of desmosomes results in loss of cohesion between keratinocytes in the epidermis, and a disruption of the barrier function served by intact skin. The process is classified as a type II hypersensitivity reaction (in which antibodies bind to antigens on the body's own tissues). On histology, the basal keratinocytes are usually still attached to the basement membrane leading to a characteristic appearance called "tombstoning". Transudative fluid accumulates in between the keratinocytes and the basal layer (suprabasal split), forming a blister and resulting in what is known as a positive Nikolsky's sign. This is a contrasting feature from bullous pemphigoid, which is thought to be due to anti-hemidesmosome antibodies, and where the detachment occurs between the epidermis and dermis (subepidermal bullae). Clinically, pemphigus vulgaris is characterized

Porphyria Cutanea Tarda -- Histopathology

Cutaneous pathologic findings: Sub-epidermal bullae and thick hyalinized blood vessels in the upper dermis are seen. Caterpillar bodies, which are linear globules that stain PAS-positive, are present at the roof of the blisters and represent disintegrating keratinocytes and basement membrane material. Dermal sclerosis may also be present, which can be histologically identical to scleroderma.

Pemphigoid

Other potential contributory factors including genetic factors, environmental exposures to infections and drugs as well as the phenomenon of epitope spreading are also known to cause bullous pemphigoid. Diagnosis Diagnosis of bullous pemphigoid includes clinical assessment, skin biopsy for histopathology and direct immunofluorescence, indirect immunofluorescence and ELISA test. Among all, direct immunofluorescence is the gold standard for diagnosis of bullous pemphigoid. Clinical assessment For patients greater than 70 years old Blistering skin disease characterized by the presence of tense blisters and erosions that occur without another identifiable cause and rarely on mucosa. Unexplained pruritus, pruritic eczematous eruptions, or urticarial plaques Histopathology Lesional tissue, preferably of an intact vesicle or the edge of an intact bulla is obtained using punch biopsy for Haemotoxylin and Eosin (H&E)staining. Typical histopathologic findings include:

Pemphigus vulgaris

Before the advent of modern treatments, mortality for the disease was close to 90%. Today, the mortality rate with treatment is between 5-15% due to the introduction of corticosteroids as primary treatment. Nevertheless, in 1998, pemphigus vulgaris was the fourth most common cause of death due to a skin disorder. The disease mainly affects middle-aged and older adults between 50–60 years old. There has historically been a higher incidence in women. Signs and symptoms Pemphigus vulgaris most commonly presents with oral blisters (buccal and palatine mucosa, especially), but also includes cutaneous blisters. Other mucosal surfaces, the conjunctiva, nose, esophagus, penis, vulva, vagina, cervix, and anus, may also be affected. Flaccid blisters over the skin are frequently seen with sparing of the skin covering the palms and soles.

Pemphigus vulgaris

Diagnosis Because it is a rare disease, diagnosis is often complicated and takes a long time. Early in the disease patients may have erosions in the mouth or blisters on the skin. These blisters can be itchy or painful. Theoretically, the blisters should demonstrate a positive Nikolsky's sign, in which the skin sloughs off from slight rubbing, but this is not always reliable. The gold standard for diagnosis is a punch biopsy from the area around the lesion that is examined by direct immunofluorescent staining, in which cells are acantholytic, that is, lacking the normal intercellular connections that hold them together. These can also be seen on a Tzanck smear. These cells are basically rounded, nucleated keratinocytes formed due to antibody mediated damage to cell adhesion protein desmoglein.

Broad histopathologic patterns of non-glabrous skin and glabrous skin from patients with a new variant of endemic pemphigus foliaceus-part 1.

A prospective, controlled epidemiologic survey performed in El Bagre, Colombia revealed a new variant of endemic pemphigus disease, occurring in a gold mining region. The disease resembled Senear-Usher syndrome, and occurred in an endemic fashion. The aim of this study is to describe the most frequent histopathologic patterns in non-glabrous skin and in glabrous skin observed in these patients, and their clinical correlation. The study was performed on non-glabrous skin biopsies of 30 patients from the dominantly clinical affected areas (either on the chest, arms or face). Simultaneously, biopsies from the palms were obtained in 10 randomly chosen patients of the 30 total patients. The specimens were examined following hematoxylin and eosin (H&E) staining. The most common blisters observed were subcorneal, although in some cases intraspinous and subepidermal blisters were visualized. Our results showed a very heterogeneous pattern of histopathologic patterns in non-glabrous skin, which seemed to correlate with the clinical features. The most common pattern was typical pemphigus foliaceus-like, with some lupus erythematosus-like features. A non-specific, chronic dermatitis pattern prevailed in the clinically controlled patients taking daily corticosteroids. In the patients who have had the most severe and relapsing pemphigus, early sclerodermatous changes and scleredermoid alterations prevailed in their reticular dermis. In addition to the scleredermoid alterations, the reticular dermis showed a paucity of appendageal structures. On the contrary, in the palms, a similar pattern was seen in all cases, including thickening of the stratum corneum, hypergranulosis, edema in the papillary and reticular dermis and a dermal perivascular lymphocytic infiltrate. The direct immunofluorescence of the glabrous vs. the non-glabrous skin also showed some differences. We conclude that the histopathologic features of this new variant of endemic pemphigus are complex, therefore, classical histopathologic features previously described for superficial, endemic pemphigus cannot be used alone to diagnose this disease.

Pathoma_Husain

3. Thin-walled bullae rupture easily (Nikolsky sign), leading to shallow erosions with dried crust. 4. Immunofluorescence highlights IgG surrounding keratinocytes in a 'fish net' pattern. II. BULLOUS PEMPHIGOID A. Autoimmune destruction ofhemidesmosomes between basal cells and the underlying basement membrane B. Due to IgG antibody against hemidesmosome components (BP180) of the basement membrane C. Presents as blisters of the skin (Fig. 19.SA), usually in the elderly; oral mucosa is spared. 1. Basal cell layer is detached from the basement membrane (Fig. 19.SB). 2. D. Immunofluorescence highlights IgG along basement membrane (linear pattern). III. DERMATITIS HERPETIFORMIS A. Autoimmune deposition of IgA at the tips of dermal papillae B. Presents as pruritic vesicles and bullae that are grouped (herpetiform, Fig. 19.6) C. Strong association with celiac disease; resolves with gluten-free diet

Pemphigus

Pemphigus defines a group of autoimmune intraepithelial blistering diseases that are characterized by loss of normal cell-cell adhesion (acantholysis), and by the presence of pathogenic (predominantly IgG) autoantibodies reacting against epithelial adhesion molecules. Pemphigus is further divided in two major subtypes: pemphigus vulgaris (PV) and pemphigus foliaceus (PF). However, several other disorders such as IgA pemphigus, IgE pemphigus, pemphigus herpetiformis, drug-induced pemphigus, Senear Usher syndrome, and endemic pemphigus foliaceus exist, and are recognized by a dermatologist from the appearance and distribution of the skin lesions. It is also commonly diagnosed by specialists practicing otolaryngology- head and neck surgery, periodontists, oral and maxillofacial surgeons, and eye doctors, as lesions can affect the eyes and mucous membranes of the oral cavity. Intraorally, it resembles the more common diseases lichen planus and mucous membrane pemphigoid. Definitive

Polymorphous light eruption

Differential diagnosis The photosensitivity connected with lupus erythematosus is the main condition that may appear like PLE. However, the rash of lupus is inclined to be more persistent. PLE does not increase the risk of lupus. Other similar appearing conditions are solar urticaria, which has a shorter duration, the eczema-like condition, photosensitive dermatitis, and photosensitivity drug reaction. Prickly heat, which is caused by warm weather or heat is not the same as PLE. Photosensitivity is also found in some of the porphyrias. Nearly all cases of porphyria cutanea tarda exhibit blister formation on the skin within 2–4 days of light exposure. Variegate porphyria and hereditary coproporphyria can also exhibit symptoms of light-induced blisters. Classification Sunlight has been documented to trigger numerous skin conditions and the confusing terminology and categorisation previously has made the correct diagnosis and subsequent treatment difficult.

List of dog diseases

Lick granuloma also known as acral lick dermatitis, is a skin disorder in dogs resulting from an urge to lick the lower portion of the leg. The lesion from the incessant licking is a thickened, firm, oval plaque. Pemphigus is an uncommon autoimmune skin disease. The most common form in dogs is pemphigus foliaceus, which manifests as erosions and crusting of the skin and mucocutaneous junctions. Pemphigus vulgaris is more rare and manifests as blister-like lesions in the mouth and at mucocutaneous junctions. Bullous pemphigoid is most commonly seen in Dobermanns and Collies and appears as a scald-like lesion of the groin. Sebaceous adenitis is an uncommon autoimmune skin disease. Most commonly found in Akitas and Standard Poodles. Dermal fragility syndrome, also known as Ehlers–Danlos-like syndrome, is a rare condition in dogs characterized by increased skin elasticity and poor wound healing. There appears to be a genetic basis for the disease.

Porphyria cutanea tarda in pre-existent lupus erythematosus--is there an association?

In lupus erythematosus (LE), vesicles and bullae are only rarely seen. However, in some instances such efflorescences might suggest an association with distinct cutaneous diseases, including erythema multiforme, toxic epidermal necrolysis or autoimmune blistering disorders such as bullous pemphigoid, pemphigus vulgaris, and dermatitis herpetiformis Duhring. Another blistering disease that has been described in association with cutaneous and systemic LE is porphyria cutanea tarda (PCT). PCT is a metabolic disorder caused by a deficiency of the fifth enzyme in heme biosynthesis, uroporphyrinogen decarboxylase. Here, we report on a 57-year-old Caucasian woman of Dutch origin with a medical history of mild cutaneous LE who developed skin fragility, blistering skin lesions, milia, and facial hypertrichosis. Subsequent porphyrin analysis in urine and feces confirmed the suspected simultaneous manifestation of LE and PCT.

[Pseudo-late onset cutaneous parphyria in haemodialysis patients. Clinical and histological features. 9 cases (author's transl)].

In the context of a study of cutaneous problems seen in chronic renal failure patients on intermittent haemodialysis, the authors noted bullous eruptions very similar to late onset cutaneous porphyria. The bullae occur during the summer and are situated on exposed areas, in particular the head and neck. There is skin fragility and the bullae are transient, giving place to erosions and later to atrophic scarring. Even pseudo-miliary cysts have been observed. Histology reveals a sub-epidermal bulla, the basis of which consists of spiky papillary dermis. Immunofluorescence show immunoglobulin deposits in the vessels and basal membrane. All porphyrin measurements are negative. This disorder may be due to the presence of a non-porphyrinogenic photo-sensitising substance, found in certain types of plastic tubing.

Interstitial collagenase is expressed by keratinocytes that are actively involved in reepithelialization in blistering skin disease.

Migrating keratinocytes actively involved in reepithelialization in dermal wounds acquire a collagenolytic phenotype upon contact with the dermal matrix. To determine whether this phenotype is associated with repair in other forms of wounds, we assessed collagenase expression in 50 specimens representing a variety of blistering skin diseases, including subtypes of epidermolysis bullosa, porphyria cutanea tarda, bullous pemphigoid, pemphigus, transient acantholytic dermatosis, and suction blisters. Distinct from that seen in chronic ulcers or in normal healing by second intention, reepithelialization in these blistering conditions was not necessarily associated with a complete loss of basement membrane, as determined by immunostaining for type IV collagen. Collagenase mRNA was detected in the basal keratinocytes of several specimens of epidermolysis bullosa simplex (six of 10) and of pemphigus (three of seven), as well as in one quarter of transient acantholytic dermatosis samples in the presence of an intact basement membrane. In contrast, three of nine porphyria cutanea tarda, one third of epidermolysis bullosa acquisita, and one of 10 bullous pemphigoid samples had collagenase-positive basal keratinocytes with the basement membrane disrupted. The collagenase-positive lesions generally represented older blisters with evidence of epithelial regeneration. Collagenase was also expressed in suction blisters at 2 and 5 d after induction of the blister, but was shut off when the epidermis had healed. Other metalloproteinases were expressed occasionally, if at all. Our results suggest that keratinocyte migration is associated with collagenase expression and that contact of keratinocytes with the dermal matrix is not necessarily needed for collagenase induction.

Direct Immunofluorescence of Mechanobullous Epidermolysis Bullosa Acquisita, Porphyria Cutanea Tarda and Pseudoporphyria.

Mechanobullous epidermolysis bullosa acquisita (mEBA) can have a clinical presentation that is very similar to other blistering diseases, such as porphyria cutanea tarda (PCT) and pseudoporphyria. Direct immunofluorescence is an important feature in the diagnosis of mEBA, although features that overlap with PCT and pseudoporphyria have been reported. This retrospective observational study investigated whether direct immunofluorescence can discriminate mEBA from PCT and pseudoporphyria. Biopsies of 13 patients with mEBA, 10 with PCT and 10 with pseudoporphyria were included. In 7 cases of PCT and 4 of pseudoporphyria, direct immunofluorescence showed a pattern at the dermal-epidermal junction that appeared similar to the u-serrated pattern in mEBA. Vessel wall depositions were observed in all 3 diseases, but were more frequent and more intense in PCT and pseudoporphyria than in mEBA. Careful examination of direct immunofluorescence of mEBA vs. PCT and pseudoporphyria revealed different staining patterns, although overlapping features were present. Therefore, integrating all clinical and laboratory data is essential to differentiate between mEBA, PCT and pseudoporphyria.

Cutaneous pemphigus vulgaris with skin features similar to the classic mucocutaneous type: a case report and review of the literature.

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering skin disease that specifically involves oral mucosa. It was recently shown that a very small number of patients with PV show no mucous membrane involvement although they have circulating autoantibodies directed against both desmoglein (Dsg)1 and Dsg3 that are associated with histopathological suprabasal acantholysis. These cases are classed as cutaneous-type PV. We report here a case of cutaneous-type PV that occurred in a 50-year-old man. Clinical examination revealed numerous tense and spreading blisters and erosions over the patient's entire body, similar to the classic mucocutaneous-type PV. Interestingly, none of the previously reported patients with cutaneous PV had shown skin features like those of mucocutaneous PV, whereas the present case clearly demonstrated very typical clinical features similar to those in mucocutaneous PV.

Porphyria Cutanea Tarda -- History and Physical -- Cutaneous Manifestations

Photosensitivity, along with the formation of skin blisters, vesicles, bullae, and increased fragility of the skin, is seen on sun-exposed parts of the body. This most commonly involves the back of the hands, forearms, neck, face, and feet. These repeated insults to the skin later resolve, leaving behind hyper- and hypopigmented scars and milia, which are small yellowish-white keratin filled papules. [10] Since PCT is delayed blistering photosensitivity, most patients do not associate sun exposure with their skin lesions. [4] These skin lesions arise from minor trauma and sometimes may get infected, causing intense pain and inflammation. [18] The scarring may lead to contractures mimicking the skin findings of a patient with scleroderma, thus labeling this phenomenon as "pseudoscleroderma." [19] Scarring alopecia on the scalp can lead to a diffuse hair loss.

A case of autoimmune bullous dermatosis with features of pemphigus vulgaris and bullous pemphigoid.

Pleomorphic blisters, including tense bullae and annularly arranged vesicles around the erythema as well as erosive eruptions in the oral cavity, appeared on a 61-year-old woman 5 years after surgery for cholangiocellular carcinoma. A biopsy specimen from the oral cavity showed intraepidermal blisters, and those from skin lesions showed subepidermal blisters with infiltrates of eosinophils and neutrophils. The early-stage vesicles showed infiltrates along the epidermal-dermal junction, where electron microscopy disclosed disruption of the lamina densa, basal cells remaining on the dermis, and acantholytic keratinocytes among the infiltrates, but there was no cleavage of the epidermal-dermal junction at the lamina lucida. Direct immunofluorescence studies showed immune deposition at the intercellular space (ICS) and along the basement membrane zone (BMZ). Indirect immunofluorescence studies confirmed coexistence of IgG class anti-ICS and anti-BMZ antibodies. Although this case showed immunohistochemical features of bullous pemphigoid, the presence of suprabasal cleavage in the oral mucosa, acantholytic cells in the blister cavity, the deposition of IgG at the ICS of the perilesional epidermis, and circulating anti-ICS antibodies strongly suggested that this case was primarily pemphigus. The strong inflammation along the epidermal-dermal junction due to unknown factors may have modified the clinical appearance and the histopathology.

[Pseudoporphyria cutanea tarda in hemodialysis patients].

A bullous disease very similar to late onset cutaneous porphyria has been found in approximately 16 percent of the patients on intermittend hemodialysis. The bullae occur during the summer and are situated on exposed areas, particularly hands, head and neck. There is skin fragility and the bullae are transient, giving place to erosions and later to atrophic scarring. Even milia have been observed. Histology reveals a subepidermal bulla, the basis of which consists of spiky papillary dermis. Immunofluorescence shows immunoglobulin depoits in the vessels and basal membrans. All porphyrin measurements are negative. This disorder may be due to the precence of a non porphyrinogenic photosensitizing substance.

List of MeSH codes (C17)

– skin diseases, vascular – angiomatosis, bacillary – Behçet syndrome – mucocutaneous lymph node syndrome – polyarteritis nodosa – pyoderma gangrenosum – Sneddon syndrome – Takayasu's arteritis – temporal arteritis – urticaria – angioneurotic edema – vasculitis, allergic cutaneous – skin diseases, vesiculobullous – acantholysis – blister – dermatitis herpetiformis – eczema, dyshidrotic – epidermolysis bullosa – epidermolysis bullosa acquisita – epidermolysis bullosa dystrophica – epidermolysis bullosa, junctional – epidermolysis bullosa simplex – erythema multiforme – Stevens–Johnson syndrome – hydroa vacciniforme – pemphigoid, benign mucous membrane – pemphigoid, bullous – pemphigoid gestationis – pemphigus – pemphigus, benign familial – skin manifestations – skin neoplasms – sebaceous gland neoplasms – sweat gland neoplasms

Atypical Clinical and Serological Manifestation of Pemphigus Vegetans: A Case Report and Review of the Literature.

Pemphigus vegetans (PVeg) is a rare variant of pemphigus vulgaris characterized by pustules and/or papillomatous vegetations, preferentially affecting intertriginous and periorificial areas. Exceptional manifestations may be misdiagnosed resulting in delayed diagnosis and treatment. Diagnosis is confirmed by immunofluorescence and detection of anti-desmoglein (Dsg) 3 and/or anti-Dsg1 antibodies. We herein report an unusual manifestation of PVeg. At the time of first presentation, lesions were restricted to the right ring finger's tip. Although mucous membranes were initially not affected, high levels of anti-Dsg3 antibodies were detected while anti-Dsg1 and anti-desmocollin (Dsc) 1, 2, and 3 antibodies were absent. To compare our immunological findings with previous reports, all accessible Anglophone literature published since December 1988 was evaluated. We identified 52 patients suffering from PVeg, 7 of these showed anti-Dsg3 antibodies without any mucous membrane involvement. Notably, the detection of anti-Dsg1 and anti-Dsg3 antibodies does not necessarily correlate with the involvement of skin and/or mucous membranes. This might be due to more specific and complex antibody constellations in nonclassical or atypical pemphigus.

Porphyria Cutanea Tarda -- Evaluation

Skin Biopsy: This is not needed for diagnosis and may only help in excluding some other skin conditions from the cutaneous porphyrias. Subepidermal blisters and depositions in vessel walls are some of the biopsy findings seen in porphyrias. [21]