Epidermolytic hyperkeratosis

A 2-year-old boy reported with complaints of spontaneous, recurrent, excess ive blisters. accompanied by redness and scaling, since birth. The blisters ruptured after a couple of hours, leaving behind raw, exuding, dehuded are as, which healed by hyperpigmentation. No scarring was observed. Such episo des were quite regular in infancy. Subsequently, there was a perceptible de cline in these episodes with the growth of the child. In addition, the chil d had multiple, warty scales located in the groin, axillae, and neck. Examination of the skin surface revealed ruptured bullae of varying size. O n removal of the roof of a bulla, raw, exuding, erythematous, eroded areas were exposed (Fig. 1). Hyperpigmentation at the healing site was prominent. The lesions were located over the extremities and trunk. In addition, hype rkeratotic warty eruptions confined to the neck, axillae, and groin were al so identified (Figs 2 and 3). Palmoplantar keratosis and a single palmar cr ease on both hands were other interesting associations. (1,2) Hematoxylin a nd eosin stained sections of the skin showed marked hyperkeratosis, hypergr anulosis, and vacuolar degeneration of the stratum spinosum. In the dermis, there was a lymphohistiocytic infiltrate (Fig. 4).(3) Karyotype analysis w as normal. The blisters were snipped and the erosions were treated with loc al application of liquor aluminum acetate 1%. Amoxycillin and clavulanic ac id suspension (Augmentin) was administered at a dosage of 125 mg twice dail y until the lesions healed.