Open AccessDarier′s disease - Oral, general and histopathological features in a 7 year old child
Author(s) -
Sreedevi Dharman,
Arvind Muthukrishnan
Publication year - 2016
Publication title -
journal of the indian society of pedodontics and preventive dentistry/journal of indian society of pedodontics and preventive dentistry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.378
H-Index - 33
eISSN - 1998-3905
pISSN - 0970-4388
DOI - 10.4103/0970-4388.180449
Subject(s) - genodermatosis , darier's disease , medicine , dermatology , pathology , mucocutaneous zone , papule , lesion , darier disease , dyskeratosis , keratosis , asymptomatic , disease , hyperkeratosis , biology , biochemistry , gene
Darier's disease, also known as keratosis follicularis, is a rare autosomal dominant genodermatosis, manifesting clinically as hyperkeratotic, firm papule that predominates in the seborrheic areas and flexures with accompanying nail abnormalities. Heat, sweating, sunlight, and stress are exacerbating factors of the lesion. The oral lesions are asymptomatic and comprise multiple white papules in the buccal mucosa and hard palate, giving a cobblestone appearance, and it is characterized histologically by loss of desmosomal adhesion and abnormal keratinization resulting in mucocutaneous abnormalities. Pruritus, recurrent infections, and malodor can decrease the quality of life. We report a case of a 7-year-old boy with clinical and histological features of Darier's disease.