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.
Vandersteen PR, Muller SA. Erythrokeratodermia VariabilisAn Enzyme Histochemical and Ultrastructural Study. Arch Dermatol. 1971;103(4):362-370. doi:10.1001/archderm.1971.04000160012003