Darier Disease and Hailey–Hailey Disease

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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).




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



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).



Palmoplantar keratotic papules (Fig. 48.5).

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Apr 22, 2016 | Posted by in Dermatology | Comments Off on Darier Disease and Hailey–Hailey Disease

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