Darier disease (DD), also known as follicular keratosis, is a rare autosomal dominant hereditary acantholytic dermatosis caused by a mutation in the ATP2A2 gene. Skin lesions are characterized by persistent confluent papules, preferentially located on the seborrheic areas of the face, chest and back. The hands, feet and nails are also commonly affected. DD has a chronic course with frequent relapses. Their treatment is often difficult and unsatisfactory. We present the case of a 54-year-old male with a history of keratotic papules with a brownish-black color, with a foul-smelling, pruritic and ardent appearance on the back and trunk, since the age of 19, which worsen in the summer. His 27-year-old daughter has a similar picture with her skin lesions started at age of 17 in the region below the breast, back, trunk, feet and hands. After the skin biopsy, the diagnosis was confirmed and acitretin 30 mg/day was instituted. The importance of this case report is to alert health professionals that the diagnosis of DD should be considered in family members of patients, especially those in the first degree, and also in patients with follicular keratolytic dermatoses, being an important differential diagnosis of this group of patients diseases.
Cooper, S.M. and Burge, S.M. (2003) Darier’s Disease: Epidemiology, Pathophysiology, and Management. American Journal of Clinical Dermatology, 4, 97-105. https://doi.org/10.2165/00128071-200304020-00003
Burge, S.M. and Wilkinson, J.D. (1992) Doenca de Darier-White: uma Revisao dos Aspectos Clínicos em 163 Pacientes. The American Academy of Dermatology, 27, 40-50. https://doi.org/10.1016/0190-9622(92)70154-8
Karagiannidis, I., Brunner, M. and Zouboulis, C.C. (2016) Exacerbacao da Doenca da Doenca de Darier sob a terapia de Interferon com Sinais Clínicos do Liquen Nitidus. Relatórios de Casos em Dermatologia, 8, 218-223.
Linder, D., Marinello, E., Donisi, P.M., Salmaso, R., Zattra, E. and Zampetti, A. (2016) Dermatite Inframamária: Um Caso de Doenca Localizada de Darier de Início Tardio. Relatórios de Casos em Dermatologia, 8, 189-192.
Cohen, P.R. (2011) Longitudinal Erythronychia: Individual or Multiple Linear Red Bands of the Nail Plate: A Review of Clinical Features and Associated Conditions. American Journal of Clinical Dermatology, 12, 217-231. https://doi.org/10.2165/11586910-000000000-00000
Medeiros, P.M., Alves, N.R., de M Trujillo, J.M., da Silva, C.C., de Faria, P.C.P. and da Silva, R.S. (2015) Doenca de Darier Segmentar: Uma Apresentacao de Diagnóstico Difícil. Anais Brasileiros de Dermatologia, 90, 62-65. https://doi.org/10.1590/abd1806-4841.20153581
Gordon-Smith, K., Jones, L.A., Burge, S.M., Munro, C.S., Tavadia, S. and Craddock, N. (2010) The Neuropsychiatric Phenotype in Darier Disease. British Journal of Dermatology, 163, 515-522. https://doi.org/10.1111/j.1365-2133.2010.09834.x
Rubegni, P., Poggiali, S., Sbano, P., Risulo, M. and Fimiani, M. (2006) A Case of Darier’s Disease Successfully Treated with Topical Tacrolimus. Journal of the European Academy of Dermatology and Venereology, 20, 84-87. https://doi.org/10.1111/j.1468-3083.2005.01352.x