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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Peeling skin syndrome


Other Names for this Disease
  • Deciduous skin
  • Familial continuous skin peeling
  • Keratolysis exfoliativa congenita
  • Peeling skin syndrome, acral type (subtype)
  • Peeling skin syndrome, inflammatory type B (subtype)
More Names
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Overview



What is peeling skin syndrome?

How might peeling skin syndrome be treated?


What is peeling skin syndrome?

Peeling skin syndrome is a genetic disorder characterized by continual peeling of the skin. Affected individuals may also experience itching and reddening of the skin. The signs and symptoms of peeling skin syndrome usually appear soon after birth, but they may also develop later in life. There are two main subtypes of this condition, called noninflammatory type A and inflammatory type B. In some families, an acral form of peeling skin syndrome has been reported, in which skin peeling is strictly limited to the hands and feet. Peeling skin syndrome has an autosomal recessive pattern of inheritance. Mutations in the TGM5 gene have been identified in several patients with the acral form of peeling skin syndrome.[1]
Last updated: 3/30/2010

How might peeling skin syndrome be treated?

There is currently no cure for peeling skin syndrome and unfortunately, no effective treatment has been reported. Topical emollients (skin softening ointments) may help; plain petroleum jelly has been used by some individuals.[2][3] Keratolytic agents might speed up shedding and improve appearance. Treatments such as methotrexate, UVB phototherapy, isotretinoin, and oral corticosteroid therapy have been found to be ineffective in past studies.[2]
Last updated: 6/13/2011

References
  1. Skin Peeling Syndrome. Orphanet. July 2006; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=817. Accessed 3/30/2010.
  2. Kshitij Garg, Devesh Singh, Devesh Mishra. Peeling skin syndrome: Current status. Dermatology Online Journal. March 2010; 16(3):10. http://dermatology.cdlib.org/1603/case_presentations/10-00031/garg.html. Accessed 6/13/2011.
  3. Peeling Skin Syndrome. NORD. October 18, 2010; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/575/viewAbstract. Accessed 6/13/2011.