Both Darier and White independently described a rare genodermatosis.


Synonym: keratosis follicularis


Inheritance: autosomal dominant


Chromosome: 12q23-q24.1

Gene affected: ATP2A2

Mechanism of defect: impaired adhesion between keratinocytes


Clinical features:

(1) reddish-brown pruritic crusted papules in a seborrheic distribution

(2) nail changes (V-shaped nicking, longitudinal ridging, longitudinal bands)

(3) mucosal lesions

(4) verruciform (wart-like) flat papules on the dorsal surface of the feet and hands

(5) punctate keratosis on the soles and palms (palmoplantar keratoderma)


The skin lesions may become worse following:

(1) exposure to heat

(2) after sweating

(3) following sunlight exposure

(4) during pregnancy.


The keratotic papules may have a foul odor during the summer.


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