[Non-bullous erythroderma (congenital) ichthyosiforme with perinuclear shells]

A congenital dermatosis with an unusual course is described in a 5-year-old girl. During the first months of life she was found to have erythrokeratodermatic plaques, which developed into a non-bullous (congenital) ichthyosiform erythroderma (CIE) within 1 year. Since then, the clinical picture has remained stable. Histologically, the upper epidermal layers showed ballooning and vacuolization of the keratinocytes, glycogen storage, and focal parakeratotic keratinization. Conspicuous ultrastructural alterations consisted in the formation of shells of fine-filamentous, cross-linked material of unknown nature, binuclear keratinocytes, perinuclear edema formation, and the occurrence of lipid droplets in the horny layer. The n-alkane value was within the normal range. Our histological and ultrastructural findings are largely in accordance with those in a case of "congenital reticular ichthyosiform erythroderma". The disorder seems to represent a special genotype in the heterogeneous group of the non-bullous CIEs. We suggest naming it "non-bullous (congenital) ichthyosiform erythroderma with perinuclear shells".