Scleromyxedema diagnosis following unexplained encephalopathy.

CORRESPONDENCE Scleromyxedema diagnosis following unexplained encephalopathy Scleromyxedema is a rare idiopathic disorder, characterized by papules in a thickened mucinous dermis, with fi bro-blastic proliferation and monoclonal paraproteinemia [1, 2]. Typically, scleromyxedema affects middle-aged men, has a chronic progressive course and frequently pre sents extracutaneous complications that generally occur after cutaneous disease [3]. A 67-year-old man, previously healthy, presented with vertigo, nausea and gait unsteadiness. Examination disclosed slow right phase rotatory nystagmus, without ataxia. Diagnosed with a positional benign vertigo, he was discharged with symptomatic medication. Three days later he suddenly lost consciousness from which he did not recover. At admission he was in a coma, with conjugate ocular movements and decerebrating posture with noxious stimuli, without fever or meningeal signs. MRI was normal and laboratory studies unremarkable. A few hours later he had a tonic-clonic generalized seizure. EEG showed slow and irregular background activity. CSF evidenced pleocy-tosis (1800 cells, 95% neutrophils), with normal protein and glucose contents, and negative microbiologic, viro-logic and serologic studies. He was prescribed valproate, cefotaxime, ampicillin, acyclovir and dexamethasone. He started to recover (d3), awake but with incomprehensible speech, very confused and agitated, without motor defi cits; he had two more generalized seizures. LP was repeated (d7), and was completely normal. The patient slowly improved and, at day 11, he could speak and comprehend normally, but then started complex visual hallucinations: a two hectares fi eld in the wall, smoke, and animals running. This improved spontaneously, with no complaints on dismissal (d14). Six months later he presented a widespread symmetric eruption of 2-3 mm, fi rm, waxy, closely spaced pa pules predominating in the hands, forearms and forehead (fi gure 1). Physical examination also revealed diffuse er-ythema and induration of the skin, leading to decreased motility of the mouth and joints, especially the hands. Skin biopsy showed a diffuse mucin deposit in the dermis and marked fi broblastic proliferation. The most relevant results of the hematological investigation were IgG monoclonal gammopathy with λ light chains and absence of thyroid disease. Several well-described CNS manifestations in sclero-myxedema are encephalopathy, psychosis, stroke-like syndromes, seizures and, rarely, vertigo, memory loss, gait disturbance and dysarthria [3]. Also scleroderma-like lesions and Parkinson's disease have been described as possibly linked with exposure to pesticides [4]. There are reports of a specifi c " dermato-neuro " syndrome, in which fever, disturbed consciousness and seizures follow a fl u-like illness [5]. CNS symptoms usually …