The coexistence of Darier’s disease and Hailey-Hailey disease symptoms

Darier’s disease (keratosis follicularis) is an autosomal dominantly inherited genodermatosis characterized by greasy hyperkeratotic papules in seborrheic regions, nail abnormalities, and mucous membrane changes. Worldwide prevalence is estimated between 1 : 30,000 and 1 : 100,000. Darier’s disease is not apparent at birth. Usually, it starts either in the first or second decade of life. The sites of predilection are the seborrheic areas of the trunk and face, particularly the scalp margins, forehead, ears and nasolabial furrows, and scalp. The flexures, notably the anogenital region, the groins and the axillae, are often involved [1].
The distinctive lesion of Darier’s disease is a firm rather greasy crusted papule that is skin-colored, yellow-brown or brown. Coalescence of the papules produces irregular warty plaques or papillomatous masses which, in the flexures, become vegetating and malodorous. On the scalp, the heavy crusting simulates seborrhea but has a characteristic spiny feel to palpation. Loss of hair is exceptional but has occasionally been extensive and permanent. In Darier’s disease, the palms and soles may show punctuate keratoses or minute pits. Nails may display longitudinal ridges, red and white lines, and V-shaped nicks [2].
The lesions may cause persistent itchiness (pruritus). Exacerbations have been reported following exposure to UV radiation and herpes simplex infections. Spontaneous remissions do occur but the condition usually runs a chronic relapsing course with fluctuations, which may be seasonal and hormonal in origin. The general health normally remains unaffected.
Darier’s disease and Hailey-Hailey disease are often discussed together because of some similarities in their etiopathology. Both diseases are autosomal dominantly inherited genodermatosis with incomplete penetrance and are caused by abnormal epidermal calcium homeostasis. They are mapped to different chromosomal locations. Darier’s disease is caused by mutations in the ATP2A2 gene found on chromosome 12q23-24.1. Hailey-Hailey disease is caused by mutations in the ATP2C1 gene, located on chromosome 3q21–24 [3, 4].
Clinically, Hailey-Hailey disease includes recurrent eruptions of vesicles and bullae, crusted erosions and warty papules. Lesions develop mainly in areas exposed to friction such as the sides of the neck, axillae and groins. The condition usually starts in adolescence. This contrasts with patients suffering from Darier’s disease...


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