| • | 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
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