Mauzo Shakuntala H, Sulit Daryl J
University of Texas health Science Center at Houston.
Dermatol Online J. 2014 Oct 15;20(10):13030/qt9zj522bb.
Hailey-Hailey disease is a rare chronic skin disorder that is inherited in an autosomal dominant manner. The disease is characterized by development of vesicles and bullae typically in the intertriginous areas. On histology, there is widespread intraepidermal acantholysis causing the "dilapidated brick-wall" appearance. Mutations in the ATP2C1 gene, encoding for P-type Ca2+ transport ATPase, is the primary cause of the disease. The disease manifests around puberty and runs a chronic course with remissions and exacerbations. Ultraviolet light exposure, sweating, friction, stress, and cutaneous infections are the known precipitants of the disease. We report a case of a woman with recurrent flare-ups of Hailey-Hailey disease with repeated pregnancies and remission of her disease during non-pregnancy periods. To our knowledge, this is the first reported case of Hailey-Hailey disease exacerbated by pregnancy.
黑利-黑利病是一种罕见的慢性皮肤病，以常染色体显性方式遗传。该病的特征通常是在皮肤褶皱部位出现水疱和大疱。组织学上，有广泛的表皮内棘层松解，导致“破败砖墙”外观。编码P型钙转运ATP酶的ATP2C1基因突变是该病的主要病因。该病在青春期左右出现，病程呈慢性，有缓解和加重期。紫外线照射、出汗、摩擦、压力和皮肤感染是已知的诱发因素。我们报告一例女性患者，其黑利-黑利病反复复发，在多次怀孕期间病情加重，在非孕期病情缓解。据我们所知，这是首例报告的因怀孕而加重的黑利-黑利病病例。
