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.
Cram DL. Erythrokeratoderma Variabilis and Variable Circinate Erythrokeratodermas. Arch Dermatol. 1970;101(1):68-73. doi:10.1001/archderm.1970.04000010070012