Congenital ichthyosiform erythroderma (C.I.E.)--ultrastructural evidences of abnormal stratum corneum retention

Congenital ichthyosiform erythroderma (C.I.E.) is a skin disorder which has been considered as a hyperkinetic hyperkeratosis. This study was performed in order to demonstrate that C.I.E. pathogenesis includes both basal cell hyperplasia and abnormal stratum corneum retention. Electron microscopic studies were carried out on 5 cases of C.I.E., clinically and histopathologically diagnosed. The following pathological changes were found in stratum corneum and stratum granulosum: persistence of desmosomes in outer layers of stratum corneum, increased volume of intercorneocyte material, loss of lamellar pattern of Odland bodies, important variability in size and shape of keratohyaline granules, abnormal aggregation of tonofilaments. These constitute ultrastructural evidences of abnormal stratum corneum retention in C.I.E. pathogenesis.