A Rare Case of Progressive Symmetric Erythrokeratoderma in Down Syndrome

Progressive symmetric erythrokeratoderma (PSEK) is an uncommon cornification genodermatosis characterized by symmetrical, erythematous, and hyperkeratotic plaques on the extremities. The trunk is usually spared, but palms and soles may be involved. A variety of cutaneous disorders have been reported with increased frequency in individuals with Down syndrome. Common cutaneous conditions with increased prevalence in Down syndrome are alopecia areata, acne, hidradenitis suppurativa, seborrheic dermatitis, infections, and ichthyosis. Occurrence of PSEK in association with Down syndrome has not been reported in the literature. We report a rare case of PSEK for its atypical presentation on the trunk and unusual association with Down syndrome.