Hailey-Hailey disease - therapeutic trial with carbon dioxide laser

Authors

  • Vignesh Karthik Natarajan Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry
  • Sheela Kuruvila Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry

DOI:

https://doi.org/10.66344/jpad.26.3.2016.33

Keywords:

Hailey-Hailey disease, carbon dioxide laser

Abstract

Hailey-Hailey disease (HHD; MIM 169600) is a rare chronic autosomal dominant skin disorder resulting from a mutation in APT2C1, which codes for a Ca2+/Mn2+ ATPase protein (hSPCA1) in the Golgi apparatus. HHD is clinically characterized by recurrent episodes of vesicles, erosions and erythematous plaques in the intertriginous areas. Triggering factors include trauma, friction, sweating, ultraviolet light exposure, pregnancy, and menstruation. Nail changes include the presence of longitudinal leuconychia.  Histopathology shows areas of intraepidermal acantholysis causing the "dilapidated brick-wall" appearance. We present a case of HHD in a 33-year-old male. What was unique in our patient was the painful and therapy-resistant perianal involvement that led to significant morbidity. His mother also had HHD but other family members were unaffected. He had received several treatments earlier including topical antibiotics, antifungals, and corticosteroids without much improvement. We started him on carbon dioxide laser and presently he is under follow-up. We report this case because of its rarity.

Author Biographies

Vignesh Karthik Natarajan, Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry

Department of Dermatology, Venereology and Leprosy

Sheela Kuruvila, Department of Dermatology, Venereology and Leprosy, Pondicherry Institute of Medical Sciences, Pondicherry

Department of Dermatology, Venereology and Leprosy

References

Hailey H, Hailey H. Familial benign chronic pemphigus. Arch Dermatol Syphilol. 1939;39:679–85.

Sudbrak R, Brown J, Dobson-Stone C, Carter S, Ramser J, White J et al. Hailey-Hailey disease is caused by mutations in ATP2C1 encoding a novel Ca2+ pump. Hum Mol Genet. 2000;9:1131-40.

Burge SM. Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol. 1992;126:275-82.

Thapa DP, Jha AK, Pudasaini S, Kharel C, Shrestha S. Genital Hailey-Hailey disease: A case report. Our Dermatol Online. 2013;4:87-8.

LeBlanc KG Jr, Wharton JB, Sheehan DJ. Refractory Hailey-Hailey disease successfully treated with sandpaper dermabrasion. Skinmed. 2011;9:263-4.

Hunt KM, Jensen JD, Walsh SB, Helms ME, Soong VY, Jacobson ES et al. Successful treatment of refractory Hailey- Hailey disease with a 595-nm pulsed dye laser: a series of 7 cases. J Am Acad Dermatol. 2015;72:735-7.

Downloads

Published

2017-03-29

How to Cite

1.
Natarajan VK, Kuruvila S. Hailey-Hailey disease - therapeutic trial with carbon dioxide laser. J Pak Assoc Dermatol [Internet]. 2017Mar.29 [cited 2026Apr.21];26(3):257-9. Available from: https://www.jpad.com.pk/index.php/jpad/article/view/33

Issue

Vol. 26 No. 3 (2016): July - September

Section

Case Reports

License

Submission declaration

Authors retain the copyright to their work and grant the 'Journal of Pakistan Association of Dermatologists (JPAD)' the right of first publication under a Creative Commons Attribution 4.0 International (CC BY 4.0) license. This license allows others to share, adapt, and reuse the work for any purpose, including commercial use, as long as appropriate credit is given to the original authors and the journal.

By submitting a manuscript, authors confirm that the work has not been published previously (except as an abstract, lecture, or academic thesis), is not under review elsewhere, and has been approved by all authors and relevant authorities. Once accepted, the article will be openly accessible under the CC BY 4.0 license, ensuring wide dissemination and reuse with proper attribution.