We suspect spondylodiscitis - the febrile fever and the limitation of mobility of the spine with the acute course give the key -, and the indicated test is the MRI. "The most demonstrative test is magnetic resonance imaging (MRI), which is positive even in the first 2 weeks of the picture, which facilitates early diagnosis, with a sensitivity of 90%". Vertebral crushing would be seen in the simple X-ray, as well as vertebral ankylosing hyperostosis and many metastases. In addition, there is no data in the history pointing to bone metastases (oncologic history, weight loss etc).

We suspect spondylodiscitis - the febrile fever and the limitation of mobility of the spine with the acute course give the key -, and the indicated test is the MRI. "[HIDDEN]", which facilitates early diagnosis, with a sensitivity of 90%". Vertebral crushing would be seen in the simple X-ray, as well as vertebral ankylosing hyperostosis and many metastases. In addition, there is no data in the history pointing to bone metastases (oncologic history, weight loss etc).

A 70-year-old man consults for non-radiating lumbar pain of 1 week's duration, of progressive intensity, accompanied by marked limitation of spinal mobility and febrile fever. The lumbar spine X-ray shows no alterations. What diagnosis should we rule out first and with what technique?

Vertebral crush with computed tomography (CT).

Bone metastases with technetium bone scan.

Vertebral ankylosing hyperostosis with dorsolumbar spine x-ray.

Pyogenic spondylodiscitis with magnetic resonance imaging (MRI).

Radiologic Diagnosis of Spondylodiscitis, Role of Magnetic Resonance.

Study aim is to report the Magnetic Resonance Imaging (MRI) features of acute and chronic spontaneous spondylodiscitis. 57 year old female, complaining of a fever and longstanding cervical pain worsened during physical therapy. MR images were acquired using superconductive magnet 1.5 T, with the following sequences: sagittal PD and T2 TSE, sagittal T1 SE, axial PD and T2 TSE (lumbar spine), axial T2 GRE (cervical spine). Axial and sagittal T1 SE after administration of (gadolinium DTPA). Examination was reviewed by three radiologists and compared to CT findings. Patient reported cervical pain associated with fever and minimal weight loss. Blood tests were normal except hyperglycemia (DM tip II). X Ray: vertebral destruction localized at C-4 and C-5: NECT: destruction of the C-4/C-5 vertebral bodies (ventral part). MRI: Low signal of the bone marrow on T1l images, which enhanced after Gd-DTPA administration and became intermediate or high on T2 images. The steady high signal intensity of the disk on T2 images and enhancement on T1 images is typical for an acute inflammatory process. Bone Scintigrafi results: Bone changes suspicious for metastasis. Whole body CT results: apart from spine, no other significant changes. MRI is the most sensitive technique for the diagnosis of spondylodiscitis in the acute phase and comparable to CT regarding chronial stage of the disease. The present imagining essay os aimed at showing the main magnetic resonance imaging findings of tuberculous discitis.

[Radiologic diagnosis of spondylodiscitis: role of magnetic resonance].

To report the Magnetic Resonance Imaging (MRI) features of acute and chronic spontaneous spondylodiscitis as well as any typical patterns which can be useful for the differential diagnosis between pyogenic and tuberculous forms. Eleven patients affected with spontaneous spondylodiscitis were selected for the study; they were 7 men and 4 women ranging in age 33-87 years (mean: 64). We excluded the patients with iatrogenic spondylodiscitis. MR images were acquired with a superconductive magnet at 1.5, with the following sequences: sagittal PD and T2-weighted TSE, sagittal T1-weighted SE, axial PD and T2-weighted TSE for the lumbar spine, axial T2-weighted GRE for the cervical and dorsal spine and axial and sagittal T1-weighted SE after contrast agent (gadolinium DTPA) injection. MR images were reviewed by three experienced radiologists and morphological and signal intensity changes of vertebral body and disk were recorded on a standard form. In 9 patients it was possible to compare MR to CT findings. At the time of our observation all patients reported pain at the spine level, associated with fever and weight loss in 50% of cases and with increased values of the inflammatory markers. Three patients had infectious diseases in other organs and 2 were diabetics. Biopsy was performed in two cases only and demonstrated Staphylococcus aureus in one and Mycobacterium tuberculosis in the other patient. MRI allowed the correct diagnosis to be made in all cases, demonstrating the pathological involvement of the paravertebral structures and into the spinal canal earlier and more accurately than CT. A common finding in pyogenic and tuberculous spondylodiscitis was the low signal of the subcortical bone marrow on T1-weighted sagittal images, which enhanced after Gd-DTPA administration and became intermediate or high on T2-weighted images. Moreover, the steady high signal intensity of the disk on T2-weighted images and its contrast enhancement on T1-weighted images is typical for an acute inflammatory process. Based on our personal experience and literature data, we believe MRI to be the most sensitive technique for the diagnosis of spondylodiscitis in the acute phase, whereas it is comparable to CT in the chronic stage of the disease. At present MRI does not allow to differentiate pyogenic from tuberculous forms.

Unusual case of pyogenic spondylodiscitis, vertebral osteomyelitis and bilateral psoas abscesses after acupuncture: diagnosis and treatment with interventional management.

We report, for the first time, a case of pyogenic spondylodiscitis combined with vertebral osteomyelitis and bilateral psoas abscesses after acupuncture. A 60-year-old man was diagnosed with rectal cancer, and radical rectectomy and permanent colostomy were carried out. However, 3 years after the surgery the patient complained of pain in the lower back, and the symptoms worsened after seven sessions of acupuncture. Technetium 99m-labelled methylene diphosphonate ((99m)Tc-MDP) bone scintigraphy (BS) revealed abnormal uptake of (99m)Tc-MDP in the L4 and L5 vertebrae. He was admitted to our hospital because of suspected bone metastases from rectal cancer. He was diagnosed with infection based on a history of acupuncture and the findings of enhanced MRI and CT. Percutaneous lumbar discectomy (PLD), external drainage and irrigation using antibiotics were carried out to treat the L4-5 disc. Pathological analyses and bacterial culture of the resected disc confirmed infection with group C streptococcus. Postoperative antibiotic treatment resulted in significant pain relief on the third day and gradual complete relief. Considerable improvement was seen on CT and MRI at follow-up. We consider it highly likely that this patient's infection was caused by acupuncture. In patients with malignancy, abnormal uptake of (99m)Tc-MDP in BS may signify bone metastasis but can also be observed in bone infections. PLD can be used to resect diseased discs to relieve pain quickly and to prevent herniation of lumbar discs. After PLD, external drainage can be employed for abscess drainage, decompression and perfusion of antibiotics. PLD may serve as an alternative to open surgery for pyogenic spondylodiscitis.

[Huge dorsolumbar cold abscess associated with Pott's disease].

Pott's disease, or tuberculosis of the spine, is the most common osteoarticular tuberculosis. Among them, dorsolumbar impairment is predominant. The authors report the case of a patient with a huge cold lumbar abscess associated with Pott's disease. The patient is a 32-year-old man presenting dorsolumbar tumefaction associated with an alteration in his general condition and fever for three months. Treatment by "traditional healers" did not provide any improvement. He consulted for mild lumbar pain triggered by fatigue appearing one week before and after the failure of the traditional practitioner. The clinical examination found a temperature of 38.5°C, cachexia, mild lumber kyphosis and impressive, soft, painless and non inflammatory dorsolumbar bruised tumefaction, 40 cm high, 15 cm wide and 7 cm deep. He did not present any neurological signs. The dorsolumbar X-ray of the spine revealed a lesion associated with Pott's disease in the first and second lumbar vertebrae with pinching of the disc, punched-out lesions and osteocondensation. The ultrasound examination of the soft tissue revealed the presence of a laterovertebral collection of fluid diffusing in the subcutaneous region. The psoas major and the paravertebral muscles were not affected. A scan or MRI of the spine was not carried out. Examination of the tissue sample and drainage of the abscess confirmed the tubercular origin. Treatment with tuberculostatic drugs for 12 months associated with immobilisation resulted in a cure with sequelae of mild kyphoscoliosis vertebral statics.

Two cases of diskitis attributable to anaerobic bacteria in children.

Diskitis, an inflammation of the intervertebral disk, is generally attributable to Staphylococcus aureus and rarely Staphylococcus epidermidis, Kingella kingae, Enterobacteriaciae, and Streptococcus pneumoniae. In many cases, no bacterial growth is obtained from infected intervertebral discs. Although anaerobic bacteria were recovered from adults with spondylodiscitis, these organisms were not reported before from children. The recovery of anaerobic bacteria in 2 children with diskitis is reported. Patient 1. A 10-year-old male presented with 6 weeks of low back pain and 2 weeks of low-grade fever and abdominal pain. Physical examination was normal except for tenderness to percussion over the spine between thoracic vertebra 11 and lumbar vertebra 2. The patient had a temperature of 104 degrees F. Laboratory tests were within normal limits, except for erythrocyte sedimentation rate (ESR), which was 58 mm/hour. Blood culture showed no growth. Magnetic resonance imaging with gadolinium contrast revealed minimal inflammatory changes in the 12th thoracic vertebra/first lumbar vertebra disk. There was no other abnormality. A computed tomography (CT)-guided aspiration of the disk space yielded bloody material, which was sent for aerobic and anaerobic cultures. Gram stain showed numerous white blood cells and Gram-positive cocci in chains. Cultures for anaerobic bacteria yielded heavy growth of Peptostreptococcus magnus, which was susceptible to penicillin, clindamycin, and vancomycin. The patient was treated with intravenous penicillin 600 000 units every 6 hours for 3 weeks, and then oral amoxicillin, 500 mg every 6 hours for 3 weeks. The back pain resolved within 2 weeks, and the ESR returned to normal at the end of therapy. Follow-up for 3 years showed complete resolution of the infection. Patient 2. An 8-year-old boy presented with low back pain and low-grade fever, irritability, and general malaise for 10 days. He had had an upper respiratory tract infection with sore throat 27 days earlier, for which he received no therapy. The patient had a temperature of 102 degrees F, and physical examination was normal except for tenderness to percussion over the spine between the second and fourth lumbar vertebrae. Laboratory tests were normal, except for the ESR (42 mm/hour). Radiographs of the spine showed narrowing of the third to fourth lumbar vertebra disk space and irregularity of the margins of the vertebral endplates. A CT scan revealed a lytic bone lesion at lumbar vertebra 4, and bone scan showed an increase uptake of (99m)technetium at the third to fourth lumbar vertebra disk space. CT-guided aspiration of the disk space yielded cloudy nonfoul-smelling material, which was sent for aerobic and anaerobic cultures. Gram stain showed numerous white blood cells and fusiform Gram-negative bacilli. Anaerobic culture grew light growth of Fusobacterium nucleatum. The organism produced beta-lactamase and was susceptible to ticarcillin-clavulanate, clindamycin, metronidazole, and imipenem. Therapy with clindamycin 450 mg every 8 hours was given parenterally for 3 weeks and orally for 3 weeks. Back pain resolved within 2 weeks. A 2-year follow-up showed complete resolution and no recurrence. This report describes, for the first time, the isolation of anaerobic bacteria from children with diskitis. The lack of their recovery in previous reports and the absence of bacterial growth in over two third of these studies may be caused by the use of improper methods for their collection, transportation, and cultivation. Proper choice of antimicrobial therapy for diskitis can be accomplished only by identification of the causative organisms and its antimicrobial susceptibility. This is of particular importance in infections caused by anaerobic bacteria that are often resistant to antimicrobials used to empirically treat diskitis. This was the case in our second patient, who was infected by F nucleatum, which was resistant to beta-lactam antibiotics. The origin of the anaerobic bacteria causing the infection in our patient is probably of endogenous nature. The presence of abdominal pain in the first child may have been attributable to a subclinical abdominal pathothology. The preceding pharyngitis in the second patient may have been associated with a potential hematogenous spread of F nucleatum. P magnus has been associated with bone and joint infections. This report highlights the importance of obtaining disk space culture for aerobic and anaerobic bacteria from all children with diskitis. Future prospective studies are warranted to elucidate the role of anaerobic bacteria in diskitis in children.

Subdural tuberculous abscess of the lumbar spine in a patient with chronic low back pain.

Despite modern imaging methods, tuberculous abscess in the subdural space of the spine can lead to misdiagnosis and to neurogical complications development, even more up to paraplegia. We presented an extremely rare case of subdural tuberculous abscess of the lumbar (L) spine and paraparesis in imunocompetent a 49-year-old patient. A patient with chronic L syndrome and a history of intervertebral (IV) disc L3 and L5 operations got severe back pain late in July 2007. At the same time the patient had a purulent collection in the left knee, and was treated with high doses of corticosteroids and antibiotics. Then, the patient got a high fever, the amplification of pain in the L spine and the development of paraparesis. Erythrocyte sedimentation rate was 108 mm/1 h, C-reactive protein 106.0 mg/L, white blood cell (WBC) 38.4 x 09/L with a left turn. Magnetic resonance imaging (MRI) of the spine was registered expansive formation in the spinal canal, from the level of the IV disc L2 to the mid-L4 vertebral body. This finding is a "spoke" in favor of the extrusion and sequestration of IV disc L3 with the cranial and caudal migration. The patient underwent an emergency neurosurgical operation. The diagnosis of subdural staphylococcal abscess of L spine was made. According to the antibiogram antibiotic therapy was applied but without effect on the course of the disease. Control MRI of the L spine showed spondylodiscitis L3/L4, abscess collection in the spinal canal and paravertebral muscle abscess. Late in September 2007 the patient underwent needle biopsy of the L3 vertebral body guided by computed tomography and the acid-fast bacilli (AFB) were found. Tuberculostatics were introduced in the therapy. Two years later the patient was without significant personal difficulties, and with normal clinical, laboratory and morphological findings. Subdural tuberculous abscess of the spine is extremely rare manifestation of spine tuberculosis. The exact and early diagnosis and adequate treatment of atypical form of spine tuberculosis are key factors of good prognosis.

Spondylolisthesis

In adults with non-specific low back pain, strong evidence suggests medical imaging should not be done within the first six weeks. It is also suggested to avoid advanced imaging, such as CT or MRI, for adults without neurological symptoms or "red flags" in the patient's history. General recommendations for initial low back pain treatment is remaining active, avoiding twisting and bending, avoiding activities that worsen pain, avoiding bed rest, and possibly initiating a trial of non-steroidal anti-inflammatory drugs after consulting a physician. Children and adolescents with persistent low back pain may require earlier imaging and should be seen by physician. Once imaging is deemed necessary, a combination of plain radiography, computed tomography, and magnetic resonance imaging may be used. Images are most often taken of the lumbar spine due to spondylolisthesis most commonly involving the lumbar region. Images of the thoracic spine can be taken if a patient's history and physical

InternalMed_Harrison

of febrile back pain is broad, including pyelonephritis, pancreatitis, and viral syndromes. In addition, multiple noninfectious pathologies of the vertebral column, such as osteoporotic fracture, seronegative spondylitis (ankylosing spondylitis, psoriasis, reactive arthritis, enteropathic arthritis), and spinal stenosis must be considered. Imaging procedures are the most important tools not only for the diagnosis of vertebral osteomyelitis but also for the detection of pyogenic complications and alternative conditions (e.g., bone metastases or osteoporotic fractures). Plain radiography is a reasonable first step in evaluating patients without neurologic symptoms and may reveal an alternative diagnosis. Because of its low sensitivity, plain radiography generally is not helpful in acute osteomyelitis, but it can be useful in subacute or chronic cases. The gold standard is MRI, which should be performed expeditiously in patients with neurologic impairment in order to rule out a herniated

Vertebral osteomyelitis

Radiological intervention is often necessary to confirm the presence of vertebral osteomyelitis in the body. Plain-film radiological orders are necessary for all patients displaying symptoms of the disease. This diagnostic approach is often preliminary to other radiological procedures, such as magnetic resonance imaging, or MRI, computed tomography (CT) scan, fine-needle aspiration biopsy, and nuclear scintigraphy. The initial plain-film X-ray images are scanned for any indication of disc compression between two vertebrae or the degeneration of one or more vertebrae. Only when these findings are ambiguous is further testing necessary to diagnose the disease. Other radiological approaches offer more comprehensive imaging of the spinal area, but can often prove inconclusive. MRI scans do not expose the patient to radiation and are highly sensitive to changes in the size and appearance of the intervertebral discs; however, findings on the MRI scan may be confused with other conditions

[Surgical treatment of lumbar pyogenic spondylitis].

To investigate the diagnosis and effectiveness of surgical treatment for lumbar pyogenic spondylitis. Between February 2005 and June 2010, 15 cases of lumbar pyogenic spondylitis were treated. There were 10 males and 5 females with an average age of 48.6 years (range, 26-72 years). Affected segments included L2, 3 in 3 cases, L(3, 4) in 3 cases, L(4, 5) in 8 cases, and L5, S1 in 1 case. All cases had clinical manifestations of moderate or severe fever, local pain of lesion, and limitation of the spinal movement; 10 patients had nerve root symptoms, and 8 patients had symptoms of lower limb paraplegia. Leukocyte, C-reactive protein (CRP), and erythrocyte sedimentation rate (ESR) significantly increased in all cases. The preoperative X-ray and CT examinations showed no clear bone destruction; MRI showed decreased signal intensity on T1WI and increased signal intensity on T2WI, 7 cases had localized abscess formation. The patients underwent focal cleaning and bone grafting and fusion combined with internal fixation, including anterior operation in 13 cases, posterior operation in 1 case, and combined anterior and posterior operation in 1 case. The results of bacterial culture were positive in 10 cases. According to the results of intraoperative bacterial cultures, sensitive antibiotics were selected. Healing of incision by first intention was achieved in 14 cases, healing by second intention in 1 case. After 2 weeks, 1 case recurred and symptoms was reliefed after symptomatic treatment. Back pain and (or) lower limb pain were obviously improved in the other patients; body temperature recovered to normal after 3-4 days; leukocyte, ESR, and CRP significantly decreased. All the patients were followed up 12 to 70 months with an average of 35.4 months. The patients had no rejection, no fistula formation, no lumbar kyphosis, and no fixator loosening or breakage. The bony fusion time was 4-8 months. Combination of clinical manifestations, laboratory tests, and MRI can diagnose lumbar pyogenic spondylitis, of which CRP is an important indicator to determine progression of the disease, and MRI is important for diagnosis. Debridement combined with interbody fusion and internal fixation is a safe and effective treatment method.

InternalMed_Harrison

Vertebral osteomyelitis is often caused by staphylococci, but other bacteria or tuberculosis (Pott’s disease) may be responsible. The primary source of infection is usually the urinary tract, skin, or lungs. Intravenous drug use is a well-recognized risk factor. Whenever pyogenic osteomyelitis is found, the possibility of bacterial endocarditis should be considered. Back pain unrelieved by rest, spine tenderness over the involved spine segment, and an elevated ESR are the most common findings in vertebral osteomyelitis. Fever or an elevated white blood cell count is found in a minority of patients. MRI and CT are sensitive and specific for early detection of osteomyelitis; CT may be more readily available in emergency settings and better tolerated by some patients with severe back pain. The intervertebral disk can also be affected by infection (diskitis) and, very rarely, by tumor.

Lumbar spinal stenosis

Ankylosing spondylitis Diagnosis The diagnosis is based on clinical findings. Some patients can have a narrowed canal without symptoms, and do not require therapy. The normal lumbar central canal has a midsagittal diameter (front to back) greater than 13 mm, with an area of 1.45 cm2. Relative stenosis is said to exist when the anterior-posterior canal diameter measures between 10 and 13 mm. Absolute stenosis of the lumbar canal exists anatomically when the anterior-posterior measurement is 10 mm or less. Plain X-rays of the lumbar or cervical spine may or may not show spinal stenosis. The definitive diagnosis is established by either computerized tomography or magnetic resonance imaging (MRI) scanning. Identifying the presence of a narrowed canal makes the diagnosis of spinal stenosis.

[Spondylodiscitis without fever: a diagnostic challenge].

Three patients with different forms of vertebral osteomyelitis are presented, two with hematogenous infections caused by Streptococcus pneumoniae and Staphylococcus aureus and one with postsurgical infection after excision of a vertebral disc caused by coagulase-negative staphylococci. None of the patients was initially febrile, but all had localized back pain and a restricted range of movement of the vertebral column. EXAMINATIONS, DIAGNOSIS: In all three patients the MRI of the affected vertebral column was consistent with vertebral osteomyelitis. Microbiological diagnosis was made by bone biopsy in all patients and by blood cultures in two of them. Antibiotics were administered for 4-6 weeks. At follow-up two patients were without symptoms, but the third patient had persistent back pain without radiological signs of vertebral osteomyelitis. In patients with localized back pain vertebral osteomyelitis should be included in the differential diagnosis, even if there is no fever and no increase in white cell count, the erythrocyte sedimentation rate or C-reactive protein level and radiography is normal. Specific bacterial diagnosis should be made by multiple bone biopsy or blood cultures, before starting appropriate antibiotics.

Spondylolysis

Spondylolysis is typically caused by a stress fracture of the bone, and is especially common in adolescents who over-train in activities. The pars interarticularis is vulnerable to fracture during spinal hyperextension, especially when combined with rotation, or when experiencing a force during a landing. This stress fracture most commonly occurs where the concave lumbar spine transitions to the convex sacrum (L5-S1). A significant number of individuals with spondylolysis will develop spondylolisthesis, which is true for 50-81% of this population. Diagnosis There are several imaging techniques used to diagnose spondylolysis. Common imaging techniques include X-ray, MRI, Bone Scintigraphy (Bone Scan), and Computed Tomography (CT Scan).

Multifocal septic osteomyelitis mimicking skeletal metastatic disease in a patient with prostate cancer.

We present an unusual case of a 59 years old patient with prostate cancer, who was referred to our hospital with pleurodenia, low back and other sites of bone ostalgia, for bone scintiscan. The patient underwent a whole body bone scanning after the intravenous administration of 740MBq (99m)Tc-methylene diphosphonate (MDP). The main findings of the study were: increased radiotracer uptake at the T5, T9-T10 vertebrae, the head of the 11th rib and the area of the left sternoclavicular joint (SCJ), which were initially attributed to skeletal metastatic lesions. Another "hot" area in the left knee, was consistent with severe arthritis. Physical examination revealed fever up to 38.7°C, tenderness and swelling of his left knee and various painful sites. Due to persistent fever and markedly raised inflammatory markers (ESR 102mm/h, CRP 73.8mg/L, WBC 16.800 cells/μ L - neutrophils 78%, lymphocytes 15%, monocytes 5%, eosinophils 1%), the patient was further referred for a magnetic resonance (MR) scan with specific interest on the thoracic spine and the SCJ. In the sagittal short-tau inversion recovery (STIR) MR image, abnormally high signal involving both T9 and T10 vertebral bodies due to bone marrow oedema and irregularity of the endplates with focal destruction areas, were observed. The T9-T10 intervertebral disc had an abnormally high signal suggestive of "hot disc" sign and also a prevertebral soft tissue mass abutting the anterior aspect of the involved vertebral bodies. The axial T1-weighted image with fat saturation post gadolinium (Gd), revealed diffuse strong enhancement in the vertebral body, the paraspinal soft tissue mass and the adjacent right rib. Circumferential epidural enhancement indicative of intra-canal spread of the infection, was also noticed. Additional MR sequences covered the level of the SCJ. Extensive subarticular and soft tissue changes with fluid collection and bone oedema of the left SCJ were shown with the typical pattern of diffuse enhancement suggestive of septic arthritis. The MR imaging findings combined with the scintigraphic findings were consistent with subacute multifocal septic arthritis involving the axial skeleton, as a pyogenic spondylodiscitis at the T9-T10 level, the left SCJ joint and the left knee joint. Subsequently, aspiration of the SCJ and the left knee joint was performed. A purulent fluid was drained and sent to microbiology. The sample revealed 96.000 cells/μL (95% neutrophils) and methicillin-resistant Staphylococcus aureus (MRSA). The patient received intravenous vancomucin (2gr. twice a day for 14 days) and subsequently the dose was adjusted to maintain the vancomucin serum levels between 17 and 20mcg/mL. The total treatment duration was 12 weeks. Four months later the patient had fully recovered and his blood tests were normal. The patient had not been referred to an oncology department yet, as the onset of the arthritis occurred about two weeks after the diagnosis of prostate cancer. In conclusion, we present a patient with known malignancy, fever, skeletal pain and multiple bone lesions in the (99m)Tc-MDP and the MRI examination, not due to metastatic disease but to septic arthritis.

Spinal tumor

Imaging Imaging is often the next step when the diagnosis is unclear or there is greater suspicion for a serious condition that may need immediate intervention. Common types of medical imaging include X-rays, computer tomography scan (CT), Magnetic resonance imaging (MRI), myelography, and bone scans. MRI is the imaging of choice for spinal tumors. The MRI protocol that is most frequently used includes T1-weighted and T2-weighted sequences, including contrast enhanced T1-weighted sequences. Short-TI Inversion Recovery (STIR) is also commonly added to the MRI protocol for detecting spinal cord tumors. Myelography may be used as a substitute when the patient cannot undergo an MRI or it is unavailable. X-rays and CT are more commonly used to view the bony structures. They are less frequently used for spinal cord tumors, however, since they cannot reliably detect them. Bone scanning may be used as a supplementary imaging modality for tumors involving bony structures of the spine.

[Pyogenic sacroiliitis. Review of 8 personal cases and 200 cases from the literature].

Eight patients with a pyogenic infection of the sacroiliac joint are compared to 200 published cases. In all our patients the disease began with fever and immobilizing low back and buttock pain. All had bacterial growth in the blood cultures. Five out of 6 patients did not show inflammatory signs in the initial plain roentgenogram. Tc-99m scan was initially positive in 4 out of 6 patients. In 2 patients only the second scan, at 13 and 15 days respectively, was positive. All but one patient had a 4-6 week course of intravenous antibiotics. Three patients underwent surgery for abscesses or intraarticular sequestra. One patient with a small psoas abscess had only medical treatment under CT monitoring. All the patients recovered. From our observations and the literature we conclude that pyogenic sacroiliitis is often not recognized initially. Wrong diagnoses such as sepsis of unknown origin, appendicitis, discal hernia etc. can be avoided if pyogenic sacroiliitis is sought in a systematic fashion. The clinical diagnosis can be confirmed by bone scan, to be repeated at a later stage of disease (i.e. two weeks after onset) if the first examination is inconclusive.

Spondylolysis

Computed tomography Commonly known as a CT Scan or CAT scan, this form of imaging is very similar to x-ray technology but produces many more images than an x-ray does. The multiple images produce cross-sectional views not possible with an x-ray. This allows a physician or radiologist to examine the images from many more angles than an x-ray allows. For this reason the CT scan is much more accurate in detecting spondylolysis than an x-ray. Bone scintigraphy combined with CT scan is considered the gold standard which means that it is best at detecting spondylolysis. MRI MRI is a newer technique used to diagnose spondylolysis and is favorable for a few reasons. The MRI is much more accurate than the x-ray and also does not use radiation. The MRI uses powerful magnets and radio frequencies to produce very detailed images of many different densities of tissue including bone and soft tissues. Treatment

Rare case of brucellosis misdiagnosed as prostate carcinoma with lumbar vertebra metastasis: A case report.

Prostatitis caused by <iBrucella</i infection is rare and usually lacks typical lower urinary tract symptoms. However, <iBrucella</i infection can cause serum prostate-specific antigen levels to become abnormally elevated. When concurrent with lumbar vertebra infection and erosion, brucellosis can easily be misdiagnosed as prostate cancer with bone metastasis. A 45-year-old man complained of recurrent low back pain and fever for 2 wk. Magnetic resonance imaging of the lumbar vertebrae showed abnormal signs at the rear of the L4-5 vertebral body. Serum prostate-specific antigen level was 17.64 ng/mL, and positron emission tomography/computed tomography suggested the possibility of prostate cancer with liver and lumbar metastases. The patient was transferred to our department for further treatment. He experienced repeated bouts of fever and low back pain during hospitalization. Biopsy results indicated prostatitis. There was no significant increase in white blood cell count or procalcitonin levels. The <iMycobacterium tuberculosis</i smear and antibody detection results were negative. Cefoperazone sulbactam was not effective. Blood culture test results were positive for brucellosis, confirming the diagnosis of brucellosis. After oral anti-infection treatment with doxycycline and rifampicin, the body temperature gradually returned to normal, and lumbago improved. After continuous treatment for 6 mo, the patient recovered. In patients with low back pain and fever accompanied by elevated prostate-specific antigen levels and lesions of the prostate and lumbar spine, a detailed medical history and blood and urine cultures should be obtained, and attention should be given to the local epidemic infectious disease situation.

[Spondylodiscitis].

The authors present a clinical case of a female child, aged 23 months, previously healthy that 24 hours after falling down started to refuse walking and complained about back pain. She never had fever. She felt better with nonsteroidal antiinflammatory drugs, restarting the symptoms, two days after therapy suppression. At admission on hospital she presented inability to flex the lower back and loss of lumbar lordosis, being normal the remaining physical examination. There was a raise of erythrocyte sedimentation rate (ESR) and a radiological narrowing of the L1/L2 inter-vertebral disc space, a compatible image of spondylodiscitis in the MRI. Several diagnosis hipothesis have been considered, being infectious spondylodiscitis the most probable. We instituted tuberculosis therapeutic during one year and intravenous ceftriaxone for tree weeks followed by oral acetil cefuroxime (tree weeks). The spine has been immobilized with spine support. At four months disease and two months therapy, a Oerskovia xanthineolytica was isolated by intervertebral needle biopsy. A good clinical and radiological evolution has been observed. The authors stress the importance of MRI and intervertebral needle biopsy in the diagnosis of spondylodiscitis. It is also enhanced the use of MRI and ESR in the monitoring of response to the treatment.

Limited magnetic resonance imaging in low back pain instead of plain radiographs: experience with first 1000 cases.

We report our experience with the first 1000 patients with non-traumatic low back pain (LBP) without radiculopathy undergoing limited sequence magnetic resonance imaging (MRI) instead of plain radiography. Between January 1996 and December 1998, 1042 patients with low back pain unresponsive to conservative treatment were examined using a limited MR protocol comprising sagittal T1-weighted and STIR imaging. Plain radiographs were not performed. Malignancy, infection, vertebral fracture, spondylitis, pars defects and cord tumours were detected in 20%. Of the 82 osteoporotic vertebral fractures detected, 51 (62%) were recent and 31 had normal marrow signal indicating that they were old. Eighty pars defects were identified, 45(56%) had spondylolisthesis, 29(37%) were undisplaced and 6 (7%) had pars oedema only. Neoplastic disease was found in 17(8%) of which none was suspected before imaging. Benign neoplastic diseases such as vertebral AVM/haemangiomata were excluded. Twenty-one patients had a variety of disorders including ankylosing spondylitis (7), large vessel aneurysm (3), discitis (2), ovarian cyst (2), sequestered disc (2), sacral insufficiency fracture (2) and one patient each with burst fracture, retroperitoneal haematoma and a previously unsuspected horseshoe kidney. The majority of patients with LBP are best assessed clinically and imaging is usually not required. In patients with worrying symptoms, MRI with a limited protocol detects a greater number of abnormalities than previously reported studies using plain radiographs and has replaced plain radiography in our hospital. We report our experience with the first 1000 patients and highlight issues such as protocols, detection rates and communication issues.

Pyogenic arthritis of a lumbar facet joint.

We herein report the case of a 68-year-old man with diabetes who developed pyogenic arthritis of a lumbar facet joint after spinal injection. We performed magnetic resonance imaging (MRI), computed tomography (CT), technetium 99 methylene diphosphonate scintigraphy, and single photon emission computed tomography (SPECT) for this patient. MRI showed a lesion in the facet joint and no evidence of spondylodiscitis. CT showed a swelling of periarticular soft tissue around the facet joint. Bone scintigraphy showed a characteristic vertical uptake. In particular, SPECT was able to clearly confirm the location of the infection. An infection of the facet joint has only been rarely reported, but we recommended that this area should be carefully evaluated whenever a patient develops an infection of the lumbar spine after a spinal injection.

Spontaneous pyogenic vertebral osteomyelitis in nondrug users.

To analyze the clinical, microbiological, and radiologic features of patients without drug addiction suffering from spontaneous pyogenic vertebral osteomyelitis. We collected all microbiologically proved cases of pyogenic vertebral osteomyelitis seen between January 1980 and December 1999 in a teaching hospital. Patients with prior spinal instrumentation or surgery and injection drug users were excluded. Sixty-four patients, with a mean age of 59 +/- 17 years, were identified. In 29 (45%) patients, 1 or more underlying medical illnesses were found. The mean duration of symptoms before hospital admission was 48 +/- 40 days. Neurologic impairment was present in 18 (28%) patients. Staphylococcus aureus and gram-negative bacilli, mainly Escherichia coli, were the predominant etiologic agents. Blood cultures were positive in 72% (46/64) of cases. The cultures of spinal specimens obtained by x-ray-guided biopsy were positive in 52% (11/21) of cases, and those obtained by open biopsy in 75% (15/20) of cases. Plain radiography showed abnormalities in all but 7 patients. Fifty-one of 53 (96%) technetium Tc 99m diphosphonate bone scans and 40 of 44 (91%) gallium citrate Ga 67 bone scans showed increased uptake of tracers in the involved area. Paraspinal and epidural extension was found on computed tomography and/or magnetic resonance imaging in 74% (39/53) of cases; the presence of an extra-vertebral extension was not associated with the development of neurologic findings in many patients. Two patients died in relation to the infectious process and 3 relapsed; functional sequelae often were found. Spontaneous pyogenic vertebral osteomyelitis in nondrug users is a disease that affects mainly older patients suffering underlying medical illnesses. S aureus and E coli are the main causative microorganisms. Positive blood cultures frequently aided the diagnosis. Extra-vertebral extension is frequent but does not indicate a worse prognosis. Although life outcome is good, functional sequelae are common. Diagnostic delay before admission is a concern, and the physician should be alert to the possibility of this condition in patients with back or neck pain.

Spondylodiscitis as a spinal complication of transrectal ultrasound-guided needle biopsy of the prostate.

A case report. To describe the presentation of pyogenic spondylodiscitis as an iatrogenic spinal complication of a transrectal ultrasound-guided needle biopsy of the prostate (TUGNBP), despite prophylactic use of antibiotics, and discuss possible route of infection. Pyogenic spondylodiscitis is a rare complication of TUGNBP. Several similar case reports, have been previously published, including 1 by the authors of this case report; however, in the present case, spondylodiscitis occurred despite prophylactic antibiotic use. A 59-year-old man was admitted to the neurosurgery department, experiencing severe back and bilateral leg pain for 6 weeks. His neurological examination was normal. His medical and surgical histories were unremarkable, except for a TUGNBP performed 2 months ago because of the high serum levels of prostate-specific antigen levels. At the time of the biopsy, he had been given prophylactic oral antibiotic (ciprofloxacin, 500 mg twice a day) for 2 weeks. The day after biopsy, the patient experienced intermittent high fever and fatigue, and a week after biopsy, he complained of progressive back pain. After 2 weeks, whole-body bone scan with Tc99m-MDP revealed hyperactivity at the level of L4 and L5 vertebral bodies. His contrast-enhanced magnetic resonance image of the lumbar spine showed diffuse contrast enhancement vertebral bodies and intervertebral disc of L4 and L5 along with contrast-enhanced circumferential epidural mass extending from S2 to L3 levels. L4 hemilaminectomy and epidural and intradiscal abscess drainage at the L4-L5 levels were performed. The diagnosis was consistent with acute discitis with Gram (-) bacilli, and microbiological culture was positive for Escherichia coli. He received intravenous and oral antibiotics for 6 weeks. Acute pyogenic spondylodiscitis should be considered among the major complications of TUGNBP and may occur despite prophylactic antibiotic use.

<i>Enterococcus gallinarum</i> causing cervical vertebral osteomyelitis: Imagery detecting the process of rapid progression of degeneration-like change in 3 months.

We present a series of images of X-rays and MRI of vertebral osteomyelitis caused by <iEnterococcus gallinarum</i in a 65-year-old patient with persistent neck pain and fever accompanied by preceding transient biliary enzymes elevation. Images detected progression of degeneration-like changes of C5-7 in three months, which is too rapid for true degeneration and relatively slow for vertebral osteomyelitis of common pathogens. Though initial imagery evaluation detected merely degenerative change, the patient was followed up monthly because of persistent fever. Three months later, the images detected the typical imagery of vertebral osteomyelitis i.e., the destruction of vertebral bone: narrowing of intervertebral spaces with focal osteosclerosis and osteolysis on C5-7 became prominent. At this point, consultation to general internal medicine was made. With grade 3 regurgitation murmur, transthoracic echocardiography was performed and revealed 14-mm-in-diameter vegetation on aortic valve. Blood cultures detected <iEnterococcus gallinarum</i of which suspected entry was biliary tract. No previous case reports of <iEnterococcus gallinarum</i referred to vertebral osteomyelitis. While this case showed a typical clinical course of infective endocarditis, the course of progression of vertebral osteomyelitis and perhaps endocarditis was much slower comparing to common pathogens. This might reflect the relatively non-life-threatening features of this organism. <iEnterococcus gallinarum</i shows mild resistance to vancomycin and cephalosporins, initial therapy sometimes fails. <iEnterococcus gallinarum</i should be newly added to pathogenic candidates of vertebral osteomyelitis especially when feverish patients shows back or neck pain with preceding biliary tract problems.

[Pseudomonas aeruginosa osteitis of the spinal process after peridural anesthesia].

Osteitis of the posterior wall of the spinal canal is an exceptional complication after peridural anesthesia. Prognosis depends on early diagnosis based on clinical signs and imaging data. A 73-year-old man was hospitalized for lower back pain and fever of 3 weeks duration after a total hip arthroplasty performed under general anesthesia. Computed tomography and magnetic resonance imaging of the lumbar spine disclosed osteitis of the spinal processes. Local bacteriology sample evidenced Pseudomonas aeruginosa. Outcome was favorable after a 6-month treatment. Data in the French and English literature (since 1948) on bone infections following epidural anesthesia have included 5 cases of spondylodiscitis and 1 case of posterior wall osteitis. The diagnosis is suggested by the clinical presentation. Standard x-rays contribute little. Early diagnosis in the infraradiological phase can be obtained with bone scintigraphy. Computed tomography or magnetic resonance imaging are currently highly contributive to diagnosis and follow-up after treatment. Contamination may be direct or via the blood stream or result from an extension of a neighboring infectious focus. Antibiotic therapy and immobilization are indicated. Rigorous application of strict aseptic procedures during lumbar puncture and use of the epidural catheter are crucial for prevention.

Imaging of Degenerative and Infectious Conditions of the Spine.

: Imaging is important in the evaluation of patients with degenerative disease and infectious processes. There are numerous conditions that can manifest as low back pain (LBP) or neck pain in a patient, and in many cases, the cause may be multifactorial. Clinical history and physical examination are key components in the evaluation of such patients; however, physical examination has variable sensitivity and specificity. Although studies have demonstrated that uncomplicated acute LBP and/or radiculopathy are self-limited conditions that do not warrant any imaging, neuroimaging can provide clear anatomic delineation of potential causes of the patient's clinical presentation. Various professional organizations have recommendations for imaging of LBP, which generally agree that an imaging study is not indicated for patients with uncomplicated LBP or radiculopathy without a red flag (eg, neurological deficit such as major weakness or numbness in lower extremities, bowel or bladder dysfunction, saddle anesthesia, fever, history of cancer, intravenous drug use, immunosuppression, trauma, or worsening symptoms). Different imaging modalities have a complementary role in the diagnosis of pathologies affecting the spine. In this review, we discuss the standard nomenclature for lumbar disk pathology and the utility of various clinical imaging techniques in the evaluation of LBP/neck pain for potential neurosurgical management. The imaging appearance of spinal infections and potential mimics also is reviewed. Finally, we discuss advanced neuroradiological techniques that offer greater microstructural and functional information. ADC, apparent diffusion coefficientDTI, diffusion tensor imagingDWI, diffusion-weighted imagingDOM, diskitis-osteomyelitisLBP, low back painMRM, magnetic resonance myelographySNA, spinal neuroarthropathySPECT, single-positron emission computed tomographySTIR, short tau inversion recovery.

Management of lumbar spondylodiscitis developing after laparoscopic sacrohysteropexy with a mesh: A case report and review of the literature.

Lumbar spondylodiscitis is a rare and severe complication of laparoscopic sacrohysteropexy with a polypropylene mesh. However, a case of lumbar spondylodiscitis following laparoscopic sacrohysteropexy has not been reported so far. We present a case of lumbar spondylodiscitis following laparoscopic sacrohysteropexy with a mesh. We also discuss 33 cases of lumbar spondylodiscitis following sacral colpopexy and (or) rectopexy with a mesh. A 46-year-old woman with 3 previous vaginal deliveries underwent laparoscopic mesh sacrohysteropexy for stage III uterine prolapse. One month after surgery, the patient developed persistent symptoms, such as stiffness of the lumbosacral portion, low back pain (LBP), persistent swelling, pain between the right iliac crest and the buttock, inability to bend down, and pain in the right lower limb. Symptoms were alleviated by a nonsteroidal anti-inflammatory drug. However, in the last 7 days, symptoms worsened and she was unable to stand or walk. The patient had very limited leg mobility. Blood routine examination, erythrocyte sedimentation rate, C-reactive protein, and magnetic resonance imaging (MRI) of the lumbar spine indicated lumbar pyogenic spondylodiscitis. Removal of mesh and hysterectomy via laparoscopy were performed immediately, and antibiotics were given simultaneously. However, on the basis of MRI findings and persistent symptoms, debridement, laminectomy, spinal canal decompression, bone grafting, and internal fixation via pedicle screw placement were performed 5 months after laparoscopic sacrohysteropexy. All symptoms were alleviated 5 days after the operation. The patient could stand in the erect position and raise her lower limbs within 2 weeks. She could resume her normal activities within 2 months after the operation, and her X-ray appeared normal. Persistent LBP and radiating pain may be the signals of lumbar spondylodiscitis. MRI is the gold standard diagnostic examination for lumbar spondylodiscitis. Awareness of symptoms, such as LBP and radiating pain symptoms, timely diagnosis, mesh removal, and referral to orthopedists are important to prevent more severe complications. Surgical practice needs to be improved further and any other infections should be treated immediately as the most likely causes of lumbar spondylodiscitis are related to the mesh and other infections.

Enchondroma

magnetic resonance imaging (MRI) - a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. This test is done to rule out any associated abnormalities of the spinal cord and nerves. computed tomography scan (Also called a CT or CAT scan.) - a diagnostic imaging procedure that uses a combination of x-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. A CT scan shows detailed images of any part of the body, including the bones, muscles, fat, and organs. CT scans are more detailed than general x-rays.

Clinical features, diagnostic and therapeutic approaches to haematogenous vertebral osteomyelitis.

This article review the clinical features and the diagnostic approach to haematogenous vertebral osteomyelitis in order to optimise treatment strategies and follow-up assessment. Haematogenous spread is considered to be the most important route: the lumbar spine is the most common site of involvement for pyogenic infection and the thoracic spine for tuberculosis infection. The risk factors for developing haematogenous vertebral osteomyelitis are different among old people, adults and children: the literature reports that the incidence seems to be increasing in older patients. The source of infection in the elderly has been related to the use of intravenous access devices and the asymptomatic urinary infections. In young patients the increase has been correlated with the growing number of intravenous drug abusers, with endocarditis and with immigrants from areas where tuberculosis is still endemic. The onset of symptoms is typically insidious with neck or back pain often underestimated by the patient. Fever is present in 10-45% of patients. Spinal infections may cause severe neurological compromise in few cases, but mild neurological deficit, limited to one or two nerve roots, was detected in 28-35% of patients. The diagnosis of haematogenous vertebral osteomyelitis may be very difficult, as the symptoms can be sometimes not specific, vague or almost absent. The usual delay in diagnosis has been reported to be two to four months, despite the use of imaging techniques: in the early diagnosis of vertebral ostemyelitis is important the role of bone scintigraphy. The general principles for the management of spine infections are non operative, consisting of external immobilization and intravenous antibiotics, followed by oral antibiotics. Indications for surgery should be given in case of absence of clinical improvement after 2-3 weeks of intravenous antibiotics, persistent back pain and systemic effects of chronic infection and with presence or progression of neurological deficit in elderly or in cervical infection. Chronic ostemyelitis may require surgery in case of a development of biomechanical instability and/or a vertebral collapse with progressive deformity.

Copenhagen disease

Thoracolumbar spine X-rays are often used to confirm a diagnosis, and some prominent features include pinching of the anterior intervertebral disc, with fusion eventually going towards the rest of the vertebrae. Sagittal MRI imaging provides accurate scans of the spine showing non-infectious fusion. CT scans can also be used to locate issues in the vertebrae, but this form of diagnosis currently lacks a significant amount of support from the literature. 3D-CT scans can be utilized to clarify the extent of the vertebral malformations, and assist in the differential diagnosis, as multiple congenital spine defects, such as osteogenesis imperfecta, may accompany Copenhagen disease.

InternalMed_Harrison

Years may elapse before unequivocal sacroiliac abnormalities are evident on plain radiographs, and consequently, MRI is being increasingly used in diagnosing AS. Active sacroiliitis is best visualized by dynamic MRI with fat saturation, either T2-weighed turbo spin-echo sequence or short tau inversion recovery (STIR) with high resolution, or T1-weighted images with contrast enhancement. These techniques sensitively identify early intraarticular inflammation, cartilage changes, and underlying bone marrow edema in sacroiliitis (Fig. 384-1). They are also highly sensitive for evaluation of acute and chronic spinal changes (Fig. 384-2). Reduced bone mineral density can be detected by dual-energy x-ray absorptiometry of the femoral neck and the lumbar spine. Use of a lateral projection of the L3 vertebral body can prevent falsely elevated readings related to spinal ossification.


Porphyria cutanea tarda (PCT) is the most common porphyria and can present with three major clinical forms: familial, sporadic and toxic. Clinically, it is characterized by marked skin fragility with the appearance of erosions, vesicles and blisters after minimal trauma. The most frequent sites are the back of the hands and the face, i.e. the areas of greatest photoexposure. Clinically, the most important differential diagnosis should be established with pseudoporphyria or phototoxic bullous dermatitis, in which the blisters and cutaneous erosions are produced by photosensitivity, induced by drugs such as tetracyclines, sulfonamides, etc. However, unlike porphyria, porphyrin levels in serum, feces and urine are normal. The histopathological differential diagnosis should be established with those diseases showing dermal hyaline deposits, such as: hyalinosis cutis, in which unlike porphyria, PAS-positive hyaline deposits are extensive and massively occupy the deep dermis and subcutaneous cellular tissue; Milium colloid in which hyaline material, usually PAS negative expands the papillary dermis, forming characteristic clefts and fissures; and lipoid proteinosis, in which the material is deposited throughout the dermis, has a distribution not only perivascular but more extensive than in porphyria, and usually affects sweat glands.

60-year-old male patient who refers 10 days ago the appearance of blistering lesions on dorsum of 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.

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.

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

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.

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.

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.

A study of clinical, histopathological and direct immunofluorescence diagnosis in pemphigus group Utility of direct immunofluorescence.

To determine the diagnostic accordance between histopathological and direct immunofluorescence diagnosis of patients with autoimmune vesiculobullous skin diseases. The term pemphigus refers to a group of autoimmune blistering diseases mediated by auto-antibodies directed against desmoglein proteins. The differentiation between the various bullous diseases is important for treatment and prognosis. Direct immunofluorescence microscopy is still the gold standard in differentiating these diseases. Patients with clinical diagnosis of vesiculobullous dermatitis from pemphigus group were included in the study. We retrospectively analyzed histopathologic and direct immunofluorescence results from skin or mucosal samples over 15-year period. 81 patients were included. The accordance was good in pemphigus vulgaris and pemphigus herpetiformis, but low in pemphigus vegetans, pemphigus foliaceus and pemphigus erythematosus. No accordance was in Hailey-Hailey disease and Grover´s disease. Uncommon result in our analysis included: intraepidermal IgG and IgM depositions at DIF in one Grover´s disease patient. Our results confirmed the relevance of direct immunofluorescence assays as a necessary diagnostic method for the definitive diagnosis of autoimmune blistering disease in the case, where the clinical feature and the results of histopathology are not clear (Tab. 4, Fig. 5, Ref. 26).

Pemphigus vulgaris.

Pemphigus vulgaris is a chronic autoimmune bullous dermatosis that results from the production of autoantibodies against desmogleins 1 and 3. It is the most frequent and most severe form of pemphigus, occurring universally, usually between 40 and 60 years of age. It usually begins with blisters and erosions on the oral mucosa, followed by lesions on other mucous membranes and flaccid blisters on the skin, which can be disseminated. There is a clinical variant, pemphigus vegetans, which is characterized by the presence of vegetating lesions in the large folds of the skin. Clinical suspicion can be confirmed by cytological examination, histopathological examination, and direct and indirect immunofluorescence tests. The treatment is performed with systemic corticosteroids, and immunosuppressive drugs may be associated, among them azathioprine and mycophenolate mofetil. More severe cases may benefit from corticosteroids in the form of intravenous pulse therapy, and recent studies have shown a beneficial effect of rituximab, an anti-CD20 immunobiological drug. It is a chronic disease with mortality around 10%, and septicemia is the main cause of death. Patients need long-term and multidisciplinary follow-up.

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

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.

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:

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.

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.

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

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

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.

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.

InternalMed_Harrison

Pemphigus foliaceus (PF) is distinguished from PV by several features. In PF, acantholytic blisters are located high within the epidermis, usually just beneath the stratum corneum. Hence, PF is a more superficial blistering disease than PV. The distribution of lesions in the two disorders is much the same, except that in PF mucous membranes are almost always spared. Patients with PF rarely have intact blisters but rather exhibit shallow erosions associated with erythema, scale, and crust formation. Mild cases of PF resemble severe seborrheic dermatitis; severe PF may cause extensive exfoliation. Sun exposure (ultraviolet irradiation) may be an aggravating factor.

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.

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.

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]

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.

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.

[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.

Paraneoplastic pemphigus

Signs and symptoms While the presence of lesions is the denominator among patients with PNP, the characteristics of the lesions differ. The five clinical presentations of lesions associated with PNP include: "Pemphigus-like": Flaccid blister (discrete), crusts over the raw exuding skin lesions "Pemphigoid-like": Tense blister(s) on brick red erythema "Erythema multiforme-like": Severe polymorphic skin and/or mucous membrane lesions "Graft-vs.-host disease-like": Widespread lichenoid eruption with severe mucous membrane involvement "Lichen planus-like": Small red flat-topped scaly papules

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.

Transition from pemphigus foliaceus to pemphigus vulgaris: case report with literature review.

The transition between the main subtypes of pemphigus, pemphigus vulgaris (PV), and pemphigus foliaceus (PF) has rarely been reported. Moreover, the development of PV in a patient with PF is much more unusual than that of PF in a patient with PV. We report a 48-year-old man who presented with cutaneous lesions showing the typical clinical and histological features of PF. Five years later, his skin lesions became extensive and he developed oral erosions. His condition did not respond well to steroids and azathioprine. Histological examination of a vesicle disclosed suprabasal acantholysis in contrast to the subcorneal acantholysis discovered upon initial histological evaluation. Indirect immunofluorescence revealed IgG antikeratinocyte cell surface antibodies at a titer of 1:640. The titer was 1:160 at initial diagnosis. Upon immunoblotting, the patient's serum reacted with 130 kiloDalton (kDa) and 160 kDa proteins, suggesting desmoglein (Dsg) 3 and 1, respectively. We herein report an unusual case of PV that developed from PF during the disease's flare-up.

Porphyria cutanea tarda

The dermatological symptoms of PCT that include blistering and lesions on sun-exposed areas of the skin are caused by a buildup of porphyrin compounds (specifically uroporphyrinogen) close to the surface of the skin that have been oxidized by free radicals or sunlight. The oxidized porphyrins initiate degranulation of dermal mast cells, which release proteases that catabolize the surrounding proteins. This begins a cell-mediated positive feedback loop which matches the description of a type 4 delayed hypersensitivity reaction. The resulting blisters, therefore, do not appear immediately but begin to show up 2–3 days after sun exposure. Due to the highly conjugated structure of porphyrins involving alternating single and double carbon bonds, these compounds exhibit a deep purple color, resulting in the discoloration observed in the skin. Excess alcohol intake decreases hepcidin production which leads to increased iron absorption from the gut and an increase in oxidative stress. This