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Adult-onset acral peeling skin syndrome in a non-identical twin: a case report in South Africa.

Mathew R, Omole OB, Rigby J, Grayson W - Am J Case Rep (2014)

Bottom Line: 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.Skin biopsy taken from the palms confirmed peeling skin syndrome.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.

View Article: PubMed Central - PubMed

Affiliation: Family Medicine Unit, Sedibeng District Health Services, Vanderbijlpark, 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.

Case report: 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.

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Related in: MedlinePlus

Photograph of both hands. Note the erythema, swelling and remnant peeling of the skin of both hands.
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f1-amjcaserep-15-589: Photograph of both hands. Note the erythema, swelling and remnant peeling of the skin of both hands.

Mentions: Physical examination at presentation revealed ongoing exfoliation with new skin formation on the palms of the hands and soles of the feet. There were areas of thickened and keratinized skin with mild non-pitting swelling in the hands and feet (Figures 1 and 2). The mucous membranes were spared and other organ systems were normal.


Adult-onset acral peeling skin syndrome in a non-identical twin: a case report in South Africa.

Mathew R, Omole OB, Rigby J, Grayson W - Am J Case Rep (2014)

Photograph of both hands. Note the erythema, swelling and remnant peeling of the skin of both hands.
© Copyright Policy
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4319446&req=5

f1-amjcaserep-15-589: Photograph of both hands. Note the erythema, swelling and remnant peeling of the skin of both hands.
Mentions: Physical examination at presentation revealed ongoing exfoliation with new skin formation on the palms of the hands and soles of the feet. There were areas of thickened and keratinized skin with mild non-pitting swelling in the hands and feet (Figures 1 and 2). The mucous membranes were spared and other organ systems were normal.

Bottom Line: 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.Skin biopsy taken from the palms confirmed peeling skin syndrome.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.

View Article: PubMed Central - PubMed

Affiliation: Family Medicine Unit, Sedibeng District Health Services, Vanderbijlpark, 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.

Case report: 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.

Show MeSH
Related in: MedlinePlus