erythrokeratoderma variabilis

erythrokeratoderma var·i·a·bi·lis

A hereditary skin disease characterized by tough, scaly plaques associated with erythroderma that may vary daily in size, shape, and position.
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(6) Erythrokeratoderma variabilis is reported to worsen during pregnancy.
Chanarin-Dorfman syndrome mostly present as congenital ichthyosiform erythroderma but can also manifest as lamellar ichthyosis and erythrokeratoderma variabilis like picture.10 Our patient presented with nonbullous ichthyosiform erythroderma.
Progressive symmetric erythrokeratoderma is clinically different from erythrokeratoderma variabilis (EKV) first described by Mendes da Costa) its closest differential diagnosis, by well- demarcated non-migratory erythematous plaques in contrast to the migratory plaques seen in the latter.6 The erythema component of the erythrokeratoderma seems not to be relevant to populations with type IV-VI skin, as the erythema is not easily appreciated on dark skin.
Association of PSEK with palmoplantar keratoderma, ataxia and syndactyly has been reported.1 PSEK needs to be differentiated from erythrokeratoderma variabilis. Unlike in PSEK, the lesions in erythrokeratoderma variabilis fluctuate in their extent and configuration, involve the abdomen and thorax in addition to the extremities and show seasonal variation.