Korean J Dermatol.
1995 Jun;33(3):413-420.
Electron Microscopic and Immunohistochemical Comparative Studies of Bullous Congenital Ichthyosiform Erythroderma and Epidermal Nevus Histologically Showing Epidermolytic Hyperkeratosis
Abstract
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BACKGROUND: Although the histologic picture of epidermolytic hyperkeratosis is diagnostic for bullous congenital ichthyosiform erythrokerma (BCIE), it is not specific for it. It is found also in several other conditions, that is, linear epidermal nevus, epidermolytic keratisis palmaris et plantaris and epidermolytic acnthoma. Among these, BCIE is caused by mutations of the defferentiation s0pecific keratins K1 and K1-. These mutations produce a weakened cytoskeleton that is prone to collapse resulting I cell fragility and lysis. But the pathogenesis of epidermal nevus showing the similar histologic feature with BCIE is not known.
OBJECTIVE
The purpose of our study is to conpare the electron microscopic picture and the immunohistochemical features, and to find the possible pathogenesis of both diseases.
METHODS
We evaluated the clinical, histopathologic nd electron microscopic features of 5 BCIE cases and 14 epidermal nevus cases which were histologically diagnosed with epidermolytic hyperkeratosis at the department of dermatology at Wonju Christian Hospital, Shinchon Severance Hospital and Yongdong Severance Hospital, from January 1981 to June 1994. The immunohistochemical staining(PAP method) using monoclonal antibody against cytokeratin was performed on BCIE and epidermal nevus.
RESULTS
Light microscopy of both BCIE and epidermal nevus showed the same histologic changes including hyperkeratosis, increased keratohyaline granules, acanthosis and perinuclear vacuolization of upper malpighia layer. Electron mioroscopic findings in both diseases were similar. Aggregation of tonofilaments is noted in the squamous cells, but is not evident in basal cells.In immunohistochemical study of both diseases, 34betaE12 is stained in the whole epidermis and is stuonger ex0ressed in the basal layer tan suprabasal layers. LP34 staining is evident in suprabasal cell layers up to the cornified cell layer.
CONCLUSION
Electron microscopy and immunohistochemical study of both diseases showed the same finding. We thind that a defect in the differentiation specific keratins, K1 and K10 is perhaps involved in epidermal nevus histologically showing epidermolytic hyperkeratosis as in BCIE.
