Harshita Jain Author

Short Article Open Access

Darier Disease: A very rare Genodermatosis

Author(s):

Harshita Jain

Darier Disease: A very rare Genodermatosis. Darier disease (DD) or Darier-white disease, also known as Keratosis follicularis, is an autosomal dominant inherited genodermatosis caused by mutations of ATP2A2 gene. It is characterized by greasy hyperkeratotic papules in seborrheic regions, nail abnormalities and sometimes mucous membrane changes. Oral manifestations primarily affect the palatal and alveolar mucosa. A 24 year old girl presented with greyish coloured warty plaques scattered on the face, neck, ears, trunk, hands, legs, and groins. The lesions were foul smelling. Characteristic V-shaped nicking of the nails with longitudinal ridges parallel to the long axis were seen. Palmer pits were also present. On examining oral mucosa, multiple white pap... view more»

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