Late Onset Skin Peeling Syndrome.

Sir,

Skin peeling syndrome (SPS) also known as peeling skin disease is a rare autosomal recessive disorder, which usually presents after birth or in early adulthood. It is characterized by painless superficial peeling of the skin with or without vesiculation.[12] Apart from the skin shedding, the affected individuals are usually otherwise well. No effective treatment has been found so far for this condition. We report a case of late onset SPS, who presented in the fourth decade of life.

A 42-year-old Muslim male, born out of nonconsanguineous marriage presented with generalized peeling of skin since 1-year. The peeling started at the acral areas and progressed to involve the trunk in 3 months of time. The peeling was neither preceded by vesiculation nor associated with erythema. It was associated with mild pruritus. There was no seasonal variation in the lesion. There was no family history of similar complaints. The patient was diagnosed as keratolysis exfoliativa elsewhere and had received treatment in the form of mild topical keratolytic agent with no relief in the symptoms. On cutaneous examination, there was superficial peeling of skin without erythema following minimal sliding force involving mainly the upper and lower limbs and the trunk. The palms, soles and mucosa were spared. No changes in nail or hair were observed [Figures 1 and 2]. Routine laboratory investigations were normal. The only positive finding was a raised serum IgE level. Skin punch biopsy revealed focal separation of keratotic layer from the granular layer with mild dermal peri-vascular chronic inflammatory infiltrate [Figure 3].

Figure 1

Superficial peeling of skin without erythema involving upperlimb

Figure 2

Skin peeling involving upper limbs and trunk

Figure 3

Histopathology showing focal separation of keratotic layer from the granular layer with peri-vascular chronic inflammatory infiltrates

SPS is a rare genodermatosis, and only few isolated cases have been reported from India.[34] 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. SPS clinically resembles kerotolysis exfoliativa and pemphigus foliaceus. It can be well-differentiated from the above diseases by proper history and histopathology.

In our case, the patient presented with generalized type of skin peeling disorder (type A) but with severe involvement of acral areas which is a rare presentation since acral involvement in generalized type is rarely present and is usually mild. Most of the reported cases have onset in early childhood or in the first or second decade of life. Kharfi et al. reported a case of SPS in a 34-year-old female who presented with generalized painless peeling of skin since early childhood.[5] Sarma et al. 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.[15]

SPS is a rare dermatological condition, and under-reporting makes it a still more rare entity. Under-reporting could be because of nondiagnosis or mis-diagnosis as was in our case. SPS being a cosmetic disability causes emotional stress to the patient. This case has been reported so that we keep the diagnosis of SPS in mind whenever a patient presents with generalized peeling of the skin.

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