JLE

European Journal of Dermatology

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The loss of HLA-DR antigen expression by epidermal Langerhans cell in disseminated superficial porokeratosis Volume 7, issue 4, June 1997

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Department of Dermatology, Gunma University School of Medicine, 3-39-22, Showa-machi, Maebashi, Gunma, 371, Japan.

We describe a typical case of disseminated superficial porokeratosis (DSP). An immunohistochemical study demonstrated the presence of OKT-6+/HLA-DR– cells in the lesional skin, which were considered to be Langerhans cells with abnormal phenotypic expression. Porokeratosis is believed to represent a clonal expansion of mutant epidermal cells, and the systemic immunosuppressive condition is suggested to be related to the development of the disease. Our patient, however, was not considered to be under immunosuppressive conditions. The loss of HLA-DR antigen in the lesional Langerhans cells may have allowed the clonal expansion of abnormal keratinocytes in the lesion.