Erythrokeratodermia variabilis: a new case

Erythrokeratodermia variabilis: successful palliative treatment with acitretin.

Sir, Erythrokeratodermia variabilis (EKV) is a rare autosomal dominant genodermatosis which is characterized by the coexistence of hyperkeratotic plaques and transient erythematous patches. Irregular, variably shaped erythematous macular patches may enlarge or regress over time. Concurrently, there are persistent, well-demarcated, geographic, hyperkeratotic plaques which are distributed on the ...

Progressive symmetrical erythrokeratodermia - Case report*

Progressive symmetrical erythrokeratodermia is a rare autosomal dominant genodermatosis with variable penetrance described by Darier in 1911. It is characterized by erythematous and keratotic plaques, sharply defined and symmetrically distributed along the extremities, buttocks and, more rarely, on the face. We report a case of a 55-year-old patient with lesions on the dorsum of the hands, inte...

Novel mutation in GJB4 gene (connexin 30.3) in a family with erythrokeratodermia variabilis.

© 2012 The Authors. doi: 10.2340/00015555-1436 Journal Compilation © 2012 Acta Dermato-Venereologica. ISSN 0001-5555 Erythrokeratodermia variabilis (EKV, MIM: 133200) is a rare autosomal dominant disorder characterized by the association of: (i) localized or generalized hyperkeratosis, and (ii) transient erythematous areas (1). Hyperkeratotic areas show well-demarcated, geographically outlined,...

A New ELOVL4 Mutation in a Case of Spinocerebellar Ataxia With Erythrokeratodermia.

ANew ELOVL4Mutation in a Case of Spinocerebellar AtaxiaWith Erythrokeratodermia Spinocerebellar ataxia with erythrokeratodermia (SCA34; OMIM 133190) is an autosomal dominant complex form of ataxia. This condition was first described in 1972 with the report of a French-Canadian family with multiple affected individuals.1 Four decades later, a segregating locuswas identified through linkage analy...

An unusual missense mutation in the GJB3 gene resulting in severe erythrokeratodermia variabilis.