Abstract

BackgroundAcral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5. The skin peeling occurs at the separation of the stratum corneum from the stratum granulosum.Case presentationWe present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occlusion and sweating.ConclusionsSporadic cases of Acral Peeling Skin Syndrome occur in African population. There is variability in time of presentation and clinical severity even within families.

Highlights

  • Acral peeling skin syndrome is a rare autosomal recessive genodermatosis due to a missense mutation in transglutaminase 5

  • Peeling Skin Syndrome is a rare autosomal recessive skin disorder characterized by an asymptomatic superficial exfoliation due to separation of the stratum corneum [1]

  • We present a case report of two siblings with Acral Peeling Skin Syndrome (APSS)

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Summary

Background

Peeling Skin Syndrome is a rare autosomal recessive skin disorder characterized by an asymptomatic superficial exfoliation due to separation of the stratum corneum [1]. It was first described in the early twentieth century [2] and broadly classified into localized and generalized forms. Case presentation Two siblings presented with asymptomatic peeling of the hands and the feet. The younger sibling was a 6 year old girl who had peeling of the hands and feet from birth. The problem was more severe in the younger sibling than the They came from a family of five siblings. A diagnosis of APSS was made and both siblings treated with topical tretinoin 0.05% cream for four months without any improvement

Discussion
Conclusions

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