Hailey - Hailey Disease.
Keywords:
Hailey-Hailey disease, acantholysis, pathologyAbstract
Hailey – Hailey disease (familiar benign pemphigus) is a genodermatosis, inherited as an autosomal dominant condition mapped to chromosome 3q21-q24.
It encodes a Ca2+pump implicated in the production the cadherins adhesion molecules. The main histologic characteristic is the acantholysis, responsible of the intraepidermal vesicles and bullae.
We report a typical clinical and histological case of the disease.
Author Biographies
Xavier Rueda
Dermatólogo, dermatopatólogo, dermatologo oncólogo. Instituto Nacional de Cancerología, Bogotá.
Andrea Amaya
Patóloga, dermatopatóloga. Universidad Nacional de Colombia, Bogotá.
References
1. Szigeti R, Kellermayer R. Hailey Hailey disease and calcium lessons fron yeast. J Invest Dermatol. 2004;123(6):1195-6.
2. Dhitavat J, Fairclough RJ, Hovananian A, Burge SM. Calcium pumps and keratinocytes: lessons from Darier's disease and Hailey-Hailey disease. Br J Dermatol. 2004;150(5):821-8.
3. Dobson-Stone C, Fairclough R, Dunne E, Brown J, Dissarayake M, Munro C et al. Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene. J Invest Dermatol. 2002;118(2):338-43.
4. Hakuno M, Shimizu H, Akiyama M et al. Dissociation of intra- and extracellular domains of desmosomal cadherins and E-cadherin in Hailey-Hailey disease and Darier's disease. Br J Dermatol. 2000;142(4):702-11.
2. Dhitavat J, Fairclough RJ, Hovananian A, Burge SM. Calcium pumps and keratinocytes: lessons from Darier's disease and Hailey-Hailey disease. Br J Dermatol. 2004;150(5):821-8.
3. Dobson-Stone C, Fairclough R, Dunne E, Brown J, Dissarayake M, Munro C et al. Hailey-Hailey disease: molecular and clinical characterization of novel mutations in the ATP2C1 gene. J Invest Dermatol. 2002;118(2):338-43.
4. Hakuno M, Shimizu H, Akiyama M et al. Dissociation of intra- and extracellular domains of desmosomal cadherins and E-cadherin in Hailey-Hailey disease and Darier's disease. Br J Dermatol. 2000;142(4):702-11.
How to Cite
1.
Rueda X, Amaya A. Hailey - Hailey Disease. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2019 Feb. 5 [cited 2024 Jul. 3];16(1):38-9. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/95
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Published
2019-02-05
How to Cite
1.
Rueda X, Amaya A. Hailey - Hailey Disease. rev. asoc. colomb. dermatol. cir. dematol. [Internet]. 2019 Feb. 5 [cited 2024 Jul. 3];16(1):38-9. Available from: https://revista.asocolderma.org.co/index.php/asocolderma/article/view/95
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Section
Case Report
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