Unilateral segmental Darier disease following Blaschko lines: A case report

Nasza Dermatologia Online

Piotr Brzezinski¹  César Bimbi²  Katarzyna Borowska³ 

[1] 2Brazilian Society of Dermatology;1Dermatological Comittee of State Medical Council of Rio Grande do Sul Brazil;3Institute of Biology and Environmental Protection, Pomeranian Academy, Slupsk, Poland;

关键词: Darier’s disease;    Blaschko lines;    Unilateral segmental;   

DOI  :  10.7241/ourd.20173.93

来源: DOAJ

【 摘 要 】

Darier disease is an autosomal-dominant disorder of keratin production which leads to a loss in epithelial adhesion and abnormal keratinization. The clinical correspondence is keratotic papules grouped in sebaceous areas of trunk, scalp, forehead and flexures. It is a rare disease and the variant focused on here of unilateral segmental distribution following the lines of Blaschko is rarer still, considering the fact that this presentation counts for only 10% of this already uncommom disease and with only 40 cases being reported in English medical literature. Mutation in this gene is expressed in the skin and brain. The treatment of Darier disease can be challenging and is often difficult and sometimes unsatisfactory. Systemic retinoids are considered the drug of choice for treating Darier disease. However, their use is limited by potential side effects. We described the case a metalworker male with unilateral segmental Darier disease following Blaschko lines and we review the literature on this subject.

【 授权许可】

Unknown