title: Adult-onset acral peeling skin syndrome in a non-identical twin: a case report in South Africa.
abstract: BACKGROUND
  Acral peeling skin syndrome is a rare autosomal recessive disorder in which skin exfoliation is limited to the hands and feet. While it typically manifests from early childhood, in this first reported case from South Africa, the patient did not manifest clinically until the fourth decade of life.
  
  
  METHODS
  A 44-year-old woman of African descent, 1 of a set of non-identical twins, presented with recurrent episodes of skin peeling of the upper and lower limbs. The first episode occurred 4 years prior, followed by perennial skin peeling during the spring seasons. She was not on treatment for any chronic disease and reported no exposure to chemicals or other irritants. The family, including the non-identical twin sister, has no history of skin disorders and the patient's HIV antibody test was negative. At presentation, physical examination revealed ongoing exfoliation with new skin formation on the palms and soles. The mucous membranes and nails were spared. Other systems were normal. Skin biopsy taken from the palms confirmed peeling skin syndrome. The patient was managed with topical aqueous cream and analgesics. She was briefly counseled on the nature and prognosis of the disease, and referred for genetic testing and counseling. On follow-up, she continues to have skin peeling once or twice a year.
  
  
  CONCLUSIONS
  This first reported case of this rare disease in South Africa contributes to the growing body of literature on the disease and highlights the need for clinicians to be aware of its variable clinical onset.
authors.affiliation: Family Medicine Unit, Sedibeng District Health Services, Vanderbijlpark, South Africa.
authors.forename: Reshmi
authors.initials: R
authors.lastname: Mathew
authors.affiliation: Department of Family Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Olufemi B
authors.initials: OB
authors.lastname: Omole
authors.affiliation: Department of Anatomical Pathology, Charlotte Maxeke Academic Hospital, Johannesburg and the National Health Laboratory Services, Johannesburg, South Africa.
authors.forename: Jonathan
authors.initials: J
authors.lastname: Rigby
authors.affiliation: School of Pathology, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.
authors.forename: Wayne
authors.initials: W
authors.lastname: Grayson
mesh_terms: D000328:Adult; D017668:Age of Onset; D003873:Dermatitis, Exfoliative / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D010859:Pigmentation Disorders / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy; D012871:Skin Diseases / Q000151:congenital; D013019:South Africa; D014429:Twins, Dizygotic