48
Darier Disease and Hailey–Hailey Disease
Darier Disease (Keratosis Follicularis)
– 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.
– 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).
