Peeling skin syndrome: 11 cases from Saudi Arabia

Abstract

BACKGROUNDPeeling 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.METHODSTo 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.RESULTSEleven 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.CONCLUSIONAlthough 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.

Peeling skin diseases: 21 cases from Turkey and a review of the literature

“…reported eleven patients with PSS. In their studies, acral localization of PSS (33%) was more frequent than the other types 23 . In our series type A and acral PSS has occurred in six patients and type B in nine patients.…”

“…In their studies, acral localization of PSS (33%) was more frequent than the other types. 23 In our series type A and acral PSS has occurred in six patients and type B in nine patients. Type B (45%) was more prominent than the other types of PSS.…”

Peeling skin syndrome in eight cases of four different families from India and Bangladesh

“…This supported the previous observations where methotrexate, retinoids, and steroids were found to be ineffective. [25] Only two case series are published so far in the world literature; one from Saudi Arabia [26] and one from Turkey. [9] In 2011, Kose published the so far largest case series of 21 cases from Turkey.…”

Acral peeling skin syndrome

“…It includes protection of the skin against overheating, increased humidity, trauma especially in the form of pressure and friction. Some patients are relieved by the use of emollients and softening agents [15,16]. One case of successful treatment of inflammatory lesions with calcipotriol has been de-U pacjentów występowały bardziej nasilony rumień oraz głębsze złuszczanie niż w mutacji TGM5 [8].…”