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

Abstract

Peeling skin syndrome (PSS) is a rare recessively inherited ichthyosiform genodermatoses characterized by superficial skin peeling. This has 2 subtypes, acral (APSS; OMIM 609796) and generalized form (OMIM 270300). The later has been subdivided into type A (non-inflammatory) and type B (inflammatory). Eight cases of peeling skin syndrome in 4 families were recorded over a period of 5 years. They were diagnosed clinically and confirmed histopathologically. Disease onset ranged from birth to childhood age (mean 5.25 ± 4.528 years) and age at presentation ranged from 7-35 years (mean 23.25 ± 10.471 years). Males outnumbered females (M:F - 5:3). All had non-inflammatory generalized disease of type-A PSS variety, except one who had type-B PSS. Two Muslim families (1 st and 2 nd family, total 5 patients) came from nearby country Bangladesh, and the 2 Hindu families were Indian. Higher severity over acral areas in generalized type, possible autosomal dominant pattern of inheritance and improvement with age as found in this series were new manifestations and possibly unreported previously. The disease was found to be poorly responsive to oral retinoids. Prevalence of the disease may be higher than expected. Importance of mutational analysis was also highlighted.

Late onset skin peeling syndrome

“…reported a case series of 8 patients from India and Bangladesh with early onset of SPS and age at presentation being 7–35 years. [ 1 ] In contrast to the above studies, our patient reported at the age of 42 years with the onset of skin peeling at the age of 41 years, which is a late onset type of SPS. Although various studies have reported a family history of SPS, there was no family history of SPS in our patient.…”

“…It is characterized by painless superficial peeling of the skin with or without vesiculation. [ 1 2 ] Apart from the skin shedding, the affected individuals are usually otherwise well. No effective treatment has been found so far for this condition.…”

“…[ 3 4 ] SPS is divided into two forms- acral and generalized type. [ 1 ] Generalized SPS is further divided into three subtypes. Type A (noninflammatory), type B (inflammatory) and type C which starts in infancy with erythematous patches and is associated with pruritus.…”

Acral peeling skin syndrome

“…Systemic drugs include corticosteroids at low doses of 1-2 mg prednisone per kg per day for 4-6 weeks with evaluation of response and tapering of dose to prevent HPA axis suppression, growth retardation, cushingoid features, bony defects, abnormal carbohydrate and fat metabolism. Sarma N et al 8 reported that retinoid being unsuccessful in treating PSS.…”

Rarely occurring genodermatosis (acral peeling skin syndrome) – case report. Literature review of localized and generalized variants

“…Występują objawy zapalenia spojówek, warg i głębokie pęknięcia typu perléche oraz świąd. W tej odmianie brakuje badań molekularno-genetycznych [24][25][26].…”