Scleromyxedema: A Case Report and Review of the Literature
A 47-year-old Sri Lankan man had a history of slow onset, generalized, symmetric papular lesions mostly located on the face, ears, neck, upper trunk, forearms, and hands (fig. , fig. , fig. ). The papules were set close together, measuring 2–3 mm in diameter, and arranged in a linear pattern. The dorsa of both hands showed clusters of papules (fig. ). Our patient also had progressive induration, tightness, and infiltration of the skin; there was sclerodactyly, and the ‘doughnut sign’ was evident over the proximal interphalangeal joints due to infiltration of the skin (fig. ). Additionally, he started to develop progressive proximal muscle weakness, arthralgia, dyspnea especially on exertion, and regurgitation and dysphagia which were the most annoying symptoms for him at the time of examination.\nA biopsy was taken and revealed papillary dermal myxoid material (demonstrated by colloidal iron) with fibroblastic proliferation consistent with the diagnosis of scleroderma.\nLaboratory investigations revealed the following: complete blood count and differential count were within normal range; erythrocyte sedimentation rate was 17 mm/1 h (normal 0–15). Free thyroxin and thyroid stimulating hormone were within normal range. Protein electrophoresis showed the presence of a monoclonal band, about 5.2 g/l, which was typed and proved to be immunoglobulin G-lambda, after immunofixation; cholesterol 6.13 mmol/l (desirable <5.17); triglyceride 3.26 mmol/l (desirable <1.7); high-density lipoprotein 0.92 mmol/l (<1.0 low); low-density lipoprotein 4 mmol/l (desirable <3.36); glucose, urea nitrogen, serum creatinine, uric acid, total bilirubin, alkaline phosphatase, calcium, and corrected calcium were within average range; total protein 84 g/l (normal 60–80); phosphorus 1.52 mmol/l (normal 0.87–1.45); ALT (GPT) 49 U/l (normal 0–40); AST (GOT) 59 U/l (normal 0–37). It was recommended that the patient undergo radiological examination of the chest and esophagus, abdominal ultrasonography, electrocardiography, echocardiography, and electromyography of the deltoid muscle. Also, he was referred to ophthalmology, gastrointestinal, neurology, hematology and pulmonary departments for further assessment, bone marrow biopsy examination and pulmonary function tests. Unfortunately, the patient was lost to follow-up before these assessments and tests could be performed. The patient did not receive any medication due to loss to follow-up.