A 26‐year‐old white man presented with migratory peeling patches that had been present since birth. He stated that his condition was continuous and asymptomatic. There was no marked seasonal variation. Parental consanguinity was present.
His parents were first cousins, but were unaffected by peeling skin syndrome. He had four brothers and two sisters. The only affected brother, an 18‐year‐old man, had an identical history and clinical findings.
Physical examination showed widespread discrete peeling skin patches of variable size and shape with underlying erythema ( Fig. 1). The palms and soles were also involved. Peeling of the skin was produced easily by rubbing or stroking of the skin. Sheets of superficial epidermis could easily be peeled without bleeding or pain. The oral mucous
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Peeling skin patches on left arm
membrane, teeth, hair, and nails showed no abnormalities. He was in good general health.
A complete blood count, urinalysis, and routine blood chemistry were within normal limits. Serum iron and copper levels were also normal. Plasma and urinary amino acids analyzed by paper chromatography did not show deviations. There was no eosinophilia.
A skin biopsy specimen showed slight hyperkeratosis and thinning of the granular cell layer. Parakeratosis, acanthosis, and spongiosis were absent. The stratum corneum was separated from the underlying stratum granulosum. Hyperpigmentation was observed at the basal cell layer. A perivascular lymphocytic infiltrate and a few eosinophils were found in the upper dermis ( Fig. 2). Direct immunofluorescence studies did not reveal any immunoglobulin or complement deposition.
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Superficial perivascular lymphocytic infiltrate, and separation of the stratum corneum from the underlying stratum granulosum (hematoxylin and eosin, ×100)