Peeling skin syndrome is a rare genetic skin disorder characterized by continuous exfoliation of the stratum corneum. It may present at birth or in adulthood with reddening of the skin and itching. The condition is broadly classified into localized and generalized forms. The latter is further subdivided into inflammatory (type A) and non-inflammatory (type B).
The disorder is diagnosed on the basis of clinical features. There is currently no cure for peeling skin syndrome. Topical emollients and plain petroleum jelly may be used, while keratolytic medication can improve appearance through speeding up skin shedding.
Hacham-Zadeh and Holubar (1985) described two siblings of a Jewish family originating from Kurdistan who were affected by PSS. The proband, an 8-year-old boy, was referred to the skin clinic for itching, redness, and peeling of the skin. Redness, peeling of the neck, antecubital and popliteal areas, and wrists, developed four days after his birth; later on, redness and peeling of the neck, antecubital and popliteal areas, and wrists, developed four days after birth; later on redness and peeling developed all over the body. The patient's condition improved in the summer and by progression of age. The second case in the family, an 18-year-old sister, had an identical history, and clinical and laboratory findings. The patients were born to first cousins parents and had three other healthy siblings.
Abdel-Hafez at al. (1983) provided a detailed description of light and ultramicroscopic manifestations in 2 male and 1 female patients from Kuwait. The patients came from two families, one of which is consanguineous. Ultramicroscopic findings included predominance of low-density keratocytes in the corneal layer, abnormalities in distribution of lamellar granules and disrupted coating of the cells in the intercellular spaces of the corneal layer.
Kurban and Azar (1969) described a family in which three siblings born to consanguineous parents were affected by non-seasonal continuous peeling of the skin. Biospy revealed a hyperkeratotic stratum corneum with a loose structure. The authors proposed that the disorder was due to a rapid rate of profileration of the epidermis, as shown by the presence of euchromatin in the upper layer of the epidermis.
Al-Ghamdi et al. (2006) reviewed the medical records of all patients with recurring blistering diseases seen at the dermatology clinic of a hospital in Jeddah between 1986 and 2005. In this period, 11 patients (nine males, two females) were diagnosed with Peeling Skin Syndrome. All patients came from Bedouin tribes. Family history of similar condition was recorded in eight patients, while six patients had consanguineous parents. Two of the cases were brothers, while another three were also siblings of each other. The most common presentation was spontaneous localized peeling of the skin and a history of small, dry vesicles that peeled away. Skin biopsy showed either intra or subcorneal cleavage in all the cases.