Epidermolytic hyperkeratosis

A 2-year-old boy reported with complaints of spontaneous, recurrent, excessive blisters, accompanied by redness and scaling, since birth. The blisters ruptured after a couple of hours, leaving behind raw, exuding, denuded areas, which healed by hyperpigmentation. No scarring was observed. Such episodes were quite regular in infancy. Subsequently, there was a perceptible decline in these episodes with the growth of the child. In addition, the child had multiple, warty scales located in the groin, axillae, and neck. Examination of the skin surface revealed ruptured bullae of varying size. On 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, hyperkeratotic warty eruptions confined to the neck, axillae, and groin were also identified ( Figs 2 and 3 ). Palmoplantar keratosis and a single palmar crease on both hands were other interesting associations. ^{1 , 2} Hematoxylin and eosin stained sections of the skin showed marked hyperkeratosis, hypergranulosis, and vacuolar degeneration of the stratum spinosum. In the dermis, there was a lymphohistiocytic infiltrate ( Fig. 4 ). ³ Karyotype analysis was normal. The blisters were snipped and the erosions were treated with local application of liquor aluminum acetate 1%. Amoxycillin and clavulanic acid suspension (Augmentin) was administered at a dosage of 125 mg twice daily until the lesions healed.

Figure 1 Open in figure viewer PowerPoint Raw, exuding erythematous areas