A large pedigree of progressive symmetric erythrokeratoderma is reported. The proband was a 22-year-old male with generalized asymptomatic lesions characterized by symmetrical well-demarcated erythematous hyperkeratotic plaques mainly distributed on the extremities. The proband's parents were also affected, and they were first cousins. Thus, a case of familial progressive symmetric erythrokeratoderma is described.
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