Darier Disease

Darier disease or Darier-White disease is an autosomal dominantly inherited disorder characterized by greasy crusty papules in seborrheic region. These crusty patches are known as keratotic papules and also known as keratosis follicularisis.

 

Causes of Dariers disease

• Darier's is an autosomal dominant disorder with mutation in the gene ATP2A2.
• Precipitating factors include heat and humidity, mechanical trauma like friction, sunlight, and secondary bacterial infections.

Clinical features

Initial lesions are skin colored papules that develop scales or crust on its surface. The lesions gradually darken to take a brownish or blackish hue.

The distribution of the lesion is typically on the seborrheic areas: chest, back, back of ear, forehead, scalp, neck, nasolabial folds, groin, and axillae.

Oral mucosal lesion: whitish papules with central umbilications on gingiva, palate. White cobble stone white plaques may also be found.

Nails: Thinning of plate, subungual hyperkeratosis, longitudinal ridging, alternate, longitudinal white and red bands. V-shaped, triangular scalloping of distal nail plate is characteristic.

Tests and diagnosis

Family history of similar disease in parents/siblings

Skin biopsy

Prognosis

Darier's disease runs a life-long course, seasonal variations are more common.

Treatment

• Avoidance of frictions/mechanical irritations
• Use of sunscreen lotion will help to reduce the seasonal variations
• Topical retinoids: tazarotene, adapalene
• Systemic retinoids: isotretinoin or acitretin may induce temporary remission
• Topical and systemic antibiotics to treat secondary bacterial infections
• Hypertrophic scarred lesions may be treated with dermabrasion, excision and grafting, or laser excision.