|Year : 2000 | Volume
| Issue : 3 | Page : 154-155
Skin Peeling Syndrome
Rajeev Sharma, Ashok Kumar
Source of Support: None, Conflict of Interest: None
Peeling of the skin is an uncommonly encountered disorder. Occurrence of vesicles and bullae in peeling skin syndrome is very rare. We report a case of idiopathic peeling skin syndrome with vesicular lesions.
Keywords: Peeling skin syndrome
|How to cite this article:|
Sharma R, Kumar A. Skin Peeling Syndrome. Indian J Dermatol Venereol Leprol 2000;66:154-5
| Introduction|| |
Skin peeling syndrome (SPS) is an uncommonly reported disorder having onset after birth or in adulthood, characterized by spontaneous, pruritic, continual peeling of the skin with or without associated vesiculation. , The skin involvement is usually generalized, but in some patients sparing of the face, palms and soles has been reported. Histologically there is separation of the stratum corneum above the stratum granulosum.
We report a young born with SPS with vesicular lesions. There was no history of similar disease in the family or consanguinity.
| Case Report|| |
An 11-year-old boy had asymptomatic peeling of the skin and vesicular lesions on the extremities for the last 7 years. The lesions were initially limited to the hands and the feet but were gradually extending proximally to the forearms and legs. He was a full term product of non-consanguinous parents. His other three siblings were not similarly affected.
Examination showed a few, small vesicles on the left little finger and the great toes; a tense bulla on the flexor aspect of the right wrist and areas of focal peeling without erythema on the upper and lower limbs [Figure - 1]. The palms, soles and mucosal surfaces were not affected. No hair or nail changes were observed. In subsequent visits to the clinic he was found to have vesicles on the dorsum of the index and middle fingers of the left hand [Figure - 2].Laboratory investigations revealed a normal profile. Biopsy specimen taken from the peeling edge s h o w e d subcorneal split, orthokeratosis, parakeratosis, acanthosis with elongation of the rete ridges and mild superficial perivascular lymphocytic infiltrate [Figure 3] and that from the vesicle showed a sub-corneal bulla without acantholysis. Tzanck smear was negative and so was the result of bacterial culture. He was treated symptomatically with emollients that helped in amelioration of his scaling but no change was noticed in the appearance of vesiculation during a follow up period of 6 months.
| Discussion|| |
Various syndromes associated with skin peeling have been described under different names.  The paucity of cases makes the differentiation between them difficult. They are best referred to as peeling skin syndrome , to avoid confusion as they all have the same features clinically (peeling of skin) and histologically (separation of stratum corneum from stratum granulosum). Our patient also had both the cardinal features of this syndrome.
Rarely one may find associated vesiculation as described by Panja and Sengupta.  Our patient was having vesicular lesions but was asymptomatic. Panja and Sengupta's patient was symptomatic. The asymptomatic nature of the disease in our patient and appearance of vesicles on the acral parts initially led to diagnostic confusion with epidermolysis bullosa simplex superficialis (EBSS). 
They have many features in common but can be differentiated by (1) age at onset being earlier in EBSS, (2) continuous spontaneous peeling in SPS, (3) presence of mucosal lesions in EBSS, (4) hyperpigmentation on healing in SPS while atrophic scarring and milia occur in EBSS, and (5) mode of inheritance which is autosomal dominant in EBBS and recessive in SPS. Our patient had onset at 4 years of age, continuous spontaneous peeling with seasonal variations absence of mucosal lesions and healing with hyperpigmentation, which help to make a diagnosis of SPS. The management so far is only symptomatic, as everything that has been tried, has resulted in negative results. 
| References|| |
|1.||Kurban AK, Azar HA. Familial continual skin peeling. Br 3 Dermatol 1969; 81: 191 - 195. |
|2.||Panja SK, Sengupta S. Idiopathic deciduous skin. Int J Dermatol 19821; 21: 262-264. [PUBMED] |
|3.||Aras N, Sutman K, Tastan HB, et al. Peeling skin syndrome. J Am Acad Dermatol 1994; 30: 135-136. [PUBMED] |
|4.||Silverman AK, Ellis CE, Beals TF, et al. Continual skin peeling syndrome: an electron microscopic study. Arch Dermatol 1986; 122: 71-75. |
|5.||Hacham - Zodeh S, Holubar K. Skin peeling syndrome in a Kurdish family. Arch Dermatol 1985; 121: 545- 546. |
|6.||Fine JD, Johnson L, Wright T. Epidermolysis bullosa simplex superficialis: a new variant of epidermolysis bullosa characterized by subcorneal skin cleavage, mimicking peeling skin syndrome. Arch Dermatol 1989; 125: 633-638. [PUBMED] |
[Figure - 1], [Figure - 2]