Abstract
Progressive symmetric erythrokeratoderma or Gottron's syndrome is a rare autosomal dominantly inherited disorder with <100 cases reported worldwide, of which <10 are from India. It is characterized by erythematous, well-defined, hyperkeratotic plaques distributed mainly on the knees, elbows, buttocks, scalp, and dorsal surfaces of the hands and feet. In general, the trunk is spared. The condition has a progressive course during early childhood, and then is usually stable. We report a case of symmetric progressive erythrokeratoderma in two male siblings, aged 11 years and 7 years born out of second degree consanguineous marriage who presented with erythematous, scaly plaques over the extensor aspect of both extremities.
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