Acral peeling skin syndrome in two East-African siblings: case report

We present a case of two siblings who developed continuous peeling of the palms and soles from the first year of life. This peeling was more severe on the soles than palms and on younger sibling than elder sibling. Peeling is worsened by occlusion and sweating.Sporadic cases of Acral Peeling Skin Syndrome occur in African population. There is variability in time of presentation and clinical severity even within families.Peeling Skin Syndrome is a rare autosomal recessive skin disorder characterized by an asymptomatic superficial exfoliation due to separation of the stratum corneum [1]. It was first described in the early twentieth century [2] and broadly classified into localized and generalized forms. The generalized form is further subdivided into inflammatory (type A) and non-inflammatory (type B) [3]. Acral Peeling Skin Syndrome (APSS) is considered to be a localized variant [4]. We present a case report of two siblings with APSS.Two siblings presented with asymptomatic peeling of the hands and the feet. The elder sibling was a 13 year old boy who developed peeling of the skin in the first year after birth. It was noticed that the peeling of the skin left a soft red skin, which progressively thickened over a period of one to two months and turning pale before peeling again in sheets (Figure 1). The younger sibling was a 6 year old girl who had peeling of the hands and feet from birth. The problem was more severe in the younger sibling than the older sibling (Figure 2). It was associated with hyperhidrosis and became foul smelling when occluded. The peeling of the skin was aggravated by sweating, prolonged exposure to water and occlusion. There was neither associated pain nor pruritus.They came from a family of five siblings. Their parents and the other three siblings did not have a similar problem. It was a consanguineous marriage of first cousins, although no similar problem among their family members.On examination, the siblings were generally in good health w