Darier Disease and Hailey–Hailey Disease

48

Darier Disease and Hailey–Hailey Disease

Darier Disease (Keratosis Follicularis)

• Autosomal dominant.

• Mutation in the ATP2A2 gene causing dysfunction of SERCA2 protein, interfering with cellular calcium signaling.

• Clinical features.

– Onset usually between ages 6 and 20 years, with peak during adolescence.

– Chronic course, often worse in summer.

– Crusted, pink-red to brown papules that may coalesce into plaques (Fig. 48.1) in a ‘seborrheic’ and sometimes intertriginous distribution (Fig. 48.2).

Fig. 48.1 Darier disease. Truncal involvement with a predilection for seborrheic areas (A–D). Keratotic papules can vary from red (A, B) to brown (C, D) in color and may become confluent. E Severe intertriginous involvement. A, C, Courtesy, Julie V. Schaffer, MD; E, Courtesy, Daniel Hohl, MD.

Fig. 48.2 Distribution patterns for Darier, Hailey–Hailey, and Grover’s disease.

– Hypopigmented macules may be the predominant feature, especially in darker skin types (Fig. 48.3).

Fig. 48.3 Darier disease. Observed most commonly in individuals with darkly pigmented skin, a guttate leukoderma may be seen in patients with Darier disease, including the segmental form. Courtesy, Julie V. Schaffer, MD.

– Itching and malodor can be prominent.

– Flat-topped skin-colored to brown papules (resemble flat warts) on the dorsal hands and feet (Fig. 48.4).

Fig. 48.4 Darier disease. Multiple flat-topped papules on the dorsal aspect of the hand. Courtesy, Daniel Hohl, MD.

– Palmoplantar keratotic papules (Fig. 48.5).

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Apr 22, 2016 | Posted by admin in Dermatology | Comments Off

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