Clinical Features

This rare and nosologically heterogeneous disease was originally described in Japanese patients (1) but similar cases have been reported from western countries (2-4). The median age is 70 years. It occurs more frequent in males than in females (5). The original report describes extensive erythroderma, plaques and solid papules (Figs. 1,2). The coalescing brownish papules are flat-topped and observed mainly on the flexor surfaces of the extremities. The axillae, inguinal regions, antecubital and popliteal fossae, and big furrows on the abdomen are spared (the "deckchair" sign) (1,4,5). The most common abnormal laboratory findings were eosinophilia and an elevated serum IgE level (3,5). Associations with Hodgkin lymphoma (6), visceral malignancies (7), and immunodeficiency syndromes (8,9) have been reported.

Figure 1 Follicular and plaque-like infiltration of the skin on the trunk with islands of clinically uninvolved skin.

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