ICHTHYOSIFORM DERMATOSIS WITH SUPERFICIAL BLISTER FORMATION AND PEELING - EVIDENCE FOR A DESMOSOMAL ANOMALY AND ALTERED EPIDERMAL VITAMIN-AMETABOLISM

We describe a man with generalized congenital ichthyosiform dermatosis , severe cheilitis, and palmar and plantar hyperkeratosis with superfi cial blistering. Low-dose acitretin therapy induced areas of peeling s kin, similar to that seen in the peeling skin syndrome. Histologically , the skin was moderately hyperkeratotic and the palmar blisters were subcorneal. Electron microscopy revealed that the splitting occurred w ithin the desmosomal plaque. Ultrastructural and biochemical investiga tions indicated epidermal hypervitaminosis A, probably related to alte ration of epidermal retinoic acid metabolism. This disease is proposed as a hitherto unreported variant of the peeling skin syndrome.