By Disease Name > Erythrokeratoderma

Erythrokeratoderma

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a descriptive term, denotes the association of hyperkeratosis and erythema in persistent, although sometimes variable, circumscribed lesions
currently 3 well-defined syndromes can be recognized:

Erythrokeratoderma variabilis

AKA Mendes da Costas syndrome
autosomal dominant
pathogenesis: connexion gene; retention hyperkeratosis
clinical: fixed hyperkeratotic plaques with transient geographic patches of erythema (changing shape and position day to day)
ddx: figurate erythemas, symmetrical progressive erythrokeratoderma, psoriasis, parapsoriasis

Symmetrical and progressive erythrokeratoderma

AKA Gottrons syndrome
usually autosomal dominant
characterized by large fixed geographical and symmetrical fine scaly plaques of erythema with an orange hue

Progressive partially symmetric erythrokeratoderma with deafness

AKA Schnyders syndrome