Acral peeling skin syndrome

Shwayder, Tor; Conn, Sheryl; Lowe, Lori

doi:10.1001/archderm.133.4.535

Cited by 26 publications

(8 citation statements)

References 12 publications

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“…In 1987, Mevorah et al [2]reported a patient with PSS, and found 21 cases in the literature under various names (including that of Fox). Since then other cases have been reported [3, 4, 5, 6, 7, 8], yielding, to date, 30 reported cases of PSS.…”

Section: Introductionmentioning

confidence: 99%

Peeling Skin Syndrome with Hair Changes

Mevorah

Orion²,

Viragh³

et al. 1998

Dermatology

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A 13-year-old boy with typical peeling skin syndrome (PSS) is described. The clinical picture corresponded to the inflammatory variant of PSS (type B). In addition, the patient had gross and microscopic hair anomalies such as trichorrhexis invaginata-like changes, irregular hair shaft torsions and moniliform hair shaft diameter reductions. The observed dysmorphic hair changes are discussed and interpreted as being an integral component of the dermatosis in this case. To the best of our knowledge, such hair anomalies have not yet been described in PSS.

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Section: Introductionmentioning

confidence: 99%

Peeling Skin Syndrome with Hair Changes

Mevorah

Orion²,

Viragh³

et al. 1998

Dermatology

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“…Peeling skin syndrome was first described by Fox as congenital ichthyosiform erythroderma in 1921 [ 3 ]. However, in 1982, Levy and Goldsmith introduced the term “peeling skin syndrome” [ 4 ].…”

Section: Discussionmentioning

confidence: 99%

“…However, in 1982, Levy and Goldsmith introduced the term “peeling skin syndrome” [ 4 ]. There are 2 clinical variants of peeling skin syndrome, namely: Generalized and Acral peeling skin syndrome [ 3 , 5 , 6 ]. All variants are autosomal recessive and a detailed family history of skin exfoliation is therefore crucial.…”

Section: Discussionmentioning

confidence: 99%

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Adult-Onset Acral Peeling Skin Syndrome in a Non-Identical Twin: A Case Report in South Africa

Mathew¹,

Omole

Rigby

et al. 2014

Am J Case Rep

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Patient: Female, 44Final Diagnosis: Acral peeeling skin syndromeSymptoms: Recurrent skin exfoliationMedication: —Clinical Procedure: Skin biopsySpecialty: DermatologyObjective:Rare diseaseBackground: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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“…It is provoked by immersing the affected parts in water, mechanical trauma or humidity and may be more severe in summers. [3][4][5][6] Occasional painful blisters may be present. There is no definite treatment and remissions can be achieved by avoidance of water immersion, use of emollients, topical steroids, keratolytics and calcipotriol.…”

Section: Introductionmentioning

confidence: 99%