DOI: 10.36347/sjmcr.2021.v09i03.023

Pages: 279-280

Darier’s disease or keratosis follicularis is a rare autosomal dominant genodermatosis, which is characterized by greasy, crusted, keratotic, yellow brown warty papules and plaques particularly over seborrhoeic areas. Darier disease is a skin condition characterized by wart-like blemishes on the body. The blemishes are usually yellowish in color, hard to the touch, mildly greasy, and can emit a strong odor. The most common sites for blemishes are the scalp, forehead, upper arms, chest, back, knees, elbows, and behind the ear. Although this is a genetically transmitted disease according to a larger series, about 47% of patients had no clear family history, presumably because of incomplete penetrance. The prevalence of this disorder in population is 1: 100,000. The sex incidence is equal, although the males appear to be more severely affected than females. The oral mucosa is affected in 50% of the cases, and lesions are usually asymptomatic and discovered during routine dental examination. Lesions are represented by multiple firm papules with normal, whitish, or reddish color, primarily affecting the palatal and alveolar mucosa.