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April 1971

Erythrokeratodermia VariabilisAn Enzyme Histochemical and Ultrastructural Study

Author Affiliations

Rochester, Minn

From the Department of Dermatology, Mayo Graduate School of Medicine (University of Minnesota) (Dr. Vandersteen), and Mayo Clinic and Mayo Foundation (Dr. Muller), Rochester, Minn.

Arch Dermatol. 1971;103(4):362-370. doi:10.1001/archderm.1971.04000160012003

Skin biopsies done in three patients with erythrokeratodermia variabilis (EKV) seen in consecutive generations of one family were studied by enzyme histochemical and light and electron microscopic methods. Distinctive histopathologic, ultrastructural, and enzyme histochemical findings are in agreement with the thesis that erythrokeratodermia is a disease suis generis and not related to congenital ichthyosiform erythroderma. We suggest that EKV is a primary disorder of keratinization in which the basic abnormality is epidermal in origin.