Monday, December 22, 2014

DARIER’S DISEASE

DARIER’S DISEASE


Darier’s disease. Lesions may be induced by stress, heat, sweating,and maceration.

● Rare skin disorder usually transmitted in an autosomal dominant pattern.

● It is characterized by abnormal keratinocyte adhesion.

● The lesions are frequently itchy and characterized by greasy, crusted, keratotic yellow-brown papules and plaques found particularly on the scalp, forehead, ears, nasolabial folds, upper chest, back, and supraclavicular fossae.

● Lesions may be induced or exacerbated by stress, heat, sweating, and maceration (Fig.)

● Nail changes are a particularly important diagnostic feature. Longitudinal white or red streaks (often both), some of which terminate in a small nick on the free margin, are
typical fi ndings (Fig. ).


● Patients with Darier’s disease are susceptible to bacterial (particularly Staphylococcus aureus), dermatophytic, and viral infections.

● Diagnosis: skin biopsy reveals focal acantholytic dyskeratosis.

● Treatment: emollients, cool cotton clothing, topical or oral retinoids, topical 5-fl uorouracil

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