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Erythrokeratoderma Variabilis and Variable Circinate Erythrokeratodermas

David L. Cram, USAF (MC)
Arch Dermatol. 1970;101(1):68-73. doi:10.1001/archderm.1970.04000010070012.
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The variable circinate erythrokeratodermas comprise a rare group of genodermatoses having as their distinctive feature variable erythematous elements which take on bizarre shapes and are markedly influenced by environmental conditions. This is the report of a boy with features of this syndrome and a review of the literature. Erythrokeratoderma variabilis is the most frequently observed form, but other variants have been described which has led to confusion in nomenclature and classification. On present evidence a provisional grouping of these disorders seems indicated despite the fact that a consistent genetic pattern has not yet been observed. As more cases are recognized and pedigrees studied, the exact nosology of these unusual dermatoses may be clarified.


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