Fever, convulsions and coma in scleromyxedema

Scleromyxedema (SM) is a rare dermatosis characterized by a generalized papular eruption, mucin deposition in the upper dermis, and paraproteinemia. [1] The skin shows erythematous, scleroderma-like induration. [1,2] The disease primarily affects the skin, nevertheless cardiovascular, renal, and rheumatologic manifestations have been described. [1-3] Neurologic disorders associated with SM are rare but well recognized, including myopathy, entrapment neuropathy, convulsions, and encephalopathy with a grave prognosis. [4] We present a patient with SM who developed fever, convulsions, coma, and autonomic instability. A 60-year-old Arab man was previously healthy. He first noticed thickening of the skin of the face, trunk, hands, and knees 6 months prior to his admission. Two months later, numerous skin-colored papules appeared on the hands and trunk. Skin biopsy of the papules, which stained positive with colloidal iron, was compatible with SM. A week before his present admission, the patient suffered from an exacerbation of his skin disease accompanied by an upper respiratory tract infection, which resolved spontaneously. On the day of admission, the patient had a simple partial seizure followed, half an …