Erythrokeratodermia Variabilis

**Definition:** An autosomal dominant skin disease characterized by transient and variable noninflammatory ERYTHEMA and hyperkeratosis. It has been associated with mutations in the genes that code for CONNEXINS. Erythrokeratodermia variabilis inherited in an autosomal recessive fashion has also been reported. Affected individuals often develop PALMOPLANTAR KERATODERMA.

**Tree numbers:** - C16.320.850.337 - C17.800.229.606 - C17.800.428.304 - C17.800.827.337

**Synonyms:** - Mendes De Costa Syndrome - Erythrokeratodermia Variabilis with Erythema Gyratum Repens - Erythro et Keratodermia Variabilis - Erythrokeratodermia, Progressive Symmetric - Erythrokeratodermia Figurata, Congenital Familial, in Plaques - Erythrokeratodermia Figurata Variabilis