• | a descriptive term, denotes the association of hyperkeratosis and erythema in persistent, although sometimes variable, circumscribed lesions |
• | currently 3 well-defined syndrome’s can be recognized: |
Erythrokeratoderma variabilis
• | AKA Mendes da Costas syndrome |
• | 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
• | 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
|