Peeling skin syndrome (PSS) is a rare entity characterized by asymptomatic, generalized, spontaneous, and noninflammatory peeling of the skin in superficial sheets in which exfoliation of the stratum corneum occurs [1–4]. The condition begins at birth or shortly thereafter, is lifelong, and seems to have an autosomal recessive pattern of inheritance. The lesions are resistant to therapy [5]. Several anatomical, clinical variants have been described; inflammatory and noninflammatory [5], generalized or limited to acral zones [6], with eosinophilic infiltrates [7] and associated with alteration of epidermal retinoic acid metabolism [8]. Histologically, cleavage occurs within the stratum corneum or just above the stratum granulosum [4]. Ultrastructural studies have revealed blisters located either within the horny cells or in the intercellular space. Abnormalities of the lamellar bodies and keratohyalin granules have also been reported [6]. We present 2 cases of acral variant of PSS some distinctive ultrastructural features. [ABSTRACT FROM AUTHOR]