Successful Treatment of Unilateral Hailey-Hailey Disease

Main Article Content

Angelica Zambrano
Texas Tech University Health Sciences Center El Paso

Fabiola Ramirez
Adrian Guevara
Trace Worrell

Keywords

Hailey-Hailey Disease, Benign Familial Pemphigus, Superficial Radiation Therapy, Acantholysis, Blistering, Pemphigus, Laser therapy

Abstract

Hailey-Hailey Disease is a notoriously difficult to treat genodermatosis. While several treatment modalities are available, successful treatment of recalcitrant disease is not well documented. Superficial radiotherapy, a treatment typically reserved for skin cancer, has recently been utilized to treat Hailey-Hailey disease. In this case report we present the successful treatment of Hailey-Hailey disease in a patient with an unusual unilateral multifocal presentation. Based on the results of this case report, we believe SRT has strong potential to become the standard of care for recalcitrant HHD. Though there are risks to this treatment, the overall risks are minor compared to the decades of antibiotics, recurring infections, long term scarring and loss of self-esteem that arise from chronic recalcitrant HHD. 

References

1. Rogner DF, Lammer J, Zink A, Hamm H. Darier and Hailey-Hailey disease: update 2021. J Dtsch Dermatol Ges. 2021;19(10):1478-1501. doi:10.1111/ddg.14619

2. Wulf HC, Wiegell SR. Treatment of Familial Benign Chronic Pemphigus With Superficial Radiotherapy. JAMA Dermatol. 2022;158(3):283-287. doi:10.1001/jamadermatol.2021.5491

3. Adusumilli NC, Friedman AJ. Benign Familial Pemphigus. JAMA Dermatol. 2022;158(3):315. doi:10.1001/jamadermatol.2021.5177

4. Nellen RGL, Steijlen PM, van Steensel MAM, et al. Mendelian Disorders of Cornification Caused by Defects in Intracellular Calcium Pumps: Mutation Update and Database for Variants in ATP2A2 and ATP2C1 Associated with Darier Disease and Hailey-Hailey Disease. Hum Mutat. 2017;38(4):343-356. doi:10.1002/humu.23164

5. Wang Z, Li L, Sun L, et al. Review of 52 cases with Hailey-Hailey disease identified 25 novel mutations in Chinese Han population. J Dermatol. 2019;46(11):1024-1026. doi:10.1111/1346-8138.15055

6. Katzman JA, Chavan R, Holliday AC, Coman G, Grider D, Kolodney MS. Mosaic variant in ATP2C1 presenting as relapsing linear acantholytic dermatosis. Br J Dermatol. 2020;183(1):155-157. doi:10.1111/bjd.18607

7. Hwang LY, Lee JB, Richard G, Uitto JJ, Hsu S. Type 1 segmental manifestation of Hailey-Hailey disease. J Am Acad Dermatol. 2003;49(4):712-714. doi:10.1067/s0190-9622(03)00847-8

8. Higaki-Mori H, Teye K, Ishii N, Yoshida Y, Yamamoto O. Elderly-onset type 1 mosaic form of Hailey-Hailey disease with a postzygotic variant in ATP2C1. J Dermatol. 2021;48(4):e182-e183. doi:10.1111/1346-8138.15785

9. Roos DE, Reid CM. Benign familial pemphigus: little benefit from superficial radiotherapy. Australas J Dermatol. 2002;43(4):305-308. doi:10.1046/j.1440-0960.2002.00627.x

10. Campuzano-García AE, Torres-Alvarez B, Hernández-Blanco D, Castanedo-Cázares JP. Hailey-Hailey disease improved by fractional CO2 laser. J Cosmet Laser Ther. 2015;17(4):213-215. doi:10.3109/14764172.2015.1007063

11. Fitzpatrick RE. Maximizing benefits and minimizing risk with CO2 laser resurfacing. Dermatol Clin. 2002;20(1):77-86. doi:10.1016/s0733-8635(03)00047-0

12. McGregor S, Minni J, Herold D. Superficial Radiation Therapy for the Treatment of Nonmelanoma Skin Cancers. J Clin Aesthet Dermatol. 2015;8(12):12-14.

Article Sidebar

PDF
Published
Nov 13, 2023
DOI https://doi.org/10.25251/skin.7.6.18

Article Details

How to Cite
Zambrano, A., Ramirez, F., Guevara , A., & Worrell, J. (2023). Successful Treatment of Unilateral Hailey-Hailey Disease. SKIN The Journal of Cutaneous Medicine, 7(6), 1181–1185. https://doi.org/10.25251/skin.7.6.18
Issue
Vol. 7 No. 6 (2023): November 2023 Issue
Section
Brief Articles
Creative Commons License

This work is licensed under a Creative Commons Attribution 4.0 International License.

All authors retain copyright in their articles. All articles published open access allowing for immediate free access to the work and permitting any user to read, download, copy, distribute, print, search, or link to the full texts of articles, crawl them for indexing, pass them as data to software or use them for any other lawful purpose. Permitted reuse is defined by  the following user license:
 

Creative Commons Attribution (CC BY): lets others distribute and copy the article, to create extracts, abstracts, and other revised versions, adaptations or derivative works of or from an article (such as a translation), to include in a collective work (such as an anthology), to text or data mine the article, even for commercial purposes, as long as they credit the author(s), do not represent the author as endorsing their adaptation of the article, and do not modify the article in such a way as to damage the author's honor or reputation. 

Authors retain copyright in their work and license the publisher to publish the content. The publisher is the National Society for Cutaneous Medicine, with production and publishing support provided by OJS, Open Journal Systems,see https://pkp.sfu.ca/ojs/.

Prior to January 2022, authors transferred copyright to the National Society for Cutaneous Medicine. However, all articles are freely available to anyone in the world. There are no subscription fees, page charges, or article processing charges.