Peeling skin syndrome : 11 cases from Saudi Arabia

BACKGROUND: Peeling skin syndrome (PSS) is a rare genodermatoses of probable autosomal recessive inheritance. In Saudi Arabia, consanguinity of parents is common and consequently the occurrence of familial disease, including that of the skin, is not uncommon.METHODS: To characterize the clinical and pathological features of PSS in Saudi Arabia, we reviewed the medical records and clinical photographs of patients with recurring blistering diseases and conducted a histopathologic evaluation of skin biopsies to identify the site of cleavage.RESULTS: Eleven patients with PSS were seen at King Khalid National Guard Hospital in Jeddah between the years 1986 and 2005. Ages ranged between 2 and 15 years and there were 9 males (81.8%) and 2 females (18.2%). The most common presentation in the majority of patients was localized spontaneous peeling of the skin. Eight patients (72.7%) had a history of vesicles that were small, dry and peeled away. Trauma did not play a role in blister formation. All patients were local from Bedouin tribes where a family history of a similar complaint was documented in 8 cases (72.7%) and consanguinity of marriage was evident in 6 patients (54.5%). Histological examination of skin biopsies showed either intracorneal or superficial subcorneal cleavage above the granular layer in all biopsied patients.CONCLUSION: Although rare, PSS occurs in Saudi Arabia and is most likely related to consanguinity of marriages. This disease is generally mild and is characterized by intracorneal cleavage within the superficial epidermis. The disease should be recognized and not confused with other vesicobullous disease.