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Erythrokeratodermia Figurata et Variabilis


Rare syndrome determined by an autosomal dominant gene of variable expressivity with the gene locus 1p36.2p34. Characteristic lesions include well demarcated patches of erythema with changing shape and position showing a predilection for the face, buttocks and the extensor surfaces of the extremities. Other lesions consist of fixed focal hyperkeratotic plaques which also tend to appear on the extensor surfaces. The condition persists throughout life, but the general health is unaffected.


Erythrokeratodermia Figurata et Variabilis, Mendes da Costa's Syndrome, Keratitis Rubra Figurata