Peeling-Skin Syndrome: Clinical and Morphological Evidence for Two Types

Abstract

In 1921, Fox [4] very briefly reported a case of life-long, noninflammatory skin shedding under the label “keratolysis exfoliativa congenita”. Three years later, Wile [11] described an unusual type of congenital ichthyotic erythroderma occuring in three family members. He emphasized consanguinity, peeling of areas of skin, and pruritis as cardinal features of this condition. In 1982, Levy and Goldsmith [8] reported a further case and reinvestigated one of the original patients studied by Wile. They affirmed that this disease is a distinct type of congenital ichthyosis and introduced the name “peeling-skin syndrome”. It is quite obvious that the condition Wile [11] and Levy and Goldsmith [8] delineated is different from that of Fox. Unfortunately, the designation Levy and Goldsmith chose had already been used by Kurban and Azar [7], who referred to four cases similar to those of Fox as “continual skin peeling”. In the past few years both conditions have been reported as “peeling-skin syndrome” [3, 5, 9] or as “continual peeling-skin syndrome” [1, 10]. To complicate things further, both conditions are regarded as ichthyosis, and both are probably inherited as autosomal recessive traits. Therefore, I suggest distinguishing between the peeling-skin syndrome, type A (cases similar to that of Fox) and the peeling-skin syndrome, type B (cases similar to those of Wile).