LOCALIZED DARIERS-DISEASE AND ITS NOSOLOGIC STATUS

A 12-year-old girl was referred from the department of pediatrics, wit h an infected and crusted lesion on the medial aspect of the left thig h. The eruption was noticed by parents as a small red area, when the g irl was 1 day old, The pustular lesions appeared on this red area afte r 1 week. Thereafter, similar lesions erupted at the same site every y ear with the onset of summer. These lesions were reported to subside e very time within 2-3 weeks of the application of various medicaments. She was a product of normal vaginal delivery after an uncomplicated pr egnancy. There were no birth defects, Similar eruptions were not repor ted in her two siblings or her parents at the time of delivery. The ex amination of the skin revealed an erythematous, slightly raised plaque . measuring approximately 6 x 3 cm and occupying the left thigh. The p laque was covered with a multilayered, yellowish crust (Fig. 1). There were a few pustules besides a few follicular and nonfollicular kerato tic papules with greasy scales, There was very prominent epidermal atr ophy, particularly in an area of hypopigmentation, forming the lower p art of the plaque. The mucous membranes and nails were unremarkable. T he systemic examination was essentially normal. The investigations rev ealed a normal hemogram, blood urea, blood sugar, serum electrolytes, and urine examination. The roentgenograms of the chest and left thigh were also normal. The patient was treated with 1% topical framycetin. There was some improvement after 2 weeks of therapy. Sections of the b iopsy from a keratotic papule stained with hematoxylin and eosin showe d dyskeratosis in the form of 'corps ronds' at the granular layer and in the upper part of the stratum malpighii (Fig. 2). There were promin ent supra-basal clefts with focal acantholysis. In addition, there was hyperkeratosis of the epidermis and a mild subepidermal infiltrate, c onsisting predominantly of mononuclear cells.