Successful Treatment of Refractory Hailey-Hailey Disease with Narrow-Band Ultraviolet B Light: A Case Series
DOI:
https://doi.org/10.2340/actadv.v106.adv-2025-0173Keywords:
Hailey-Hailey Disease(HHD), Chronic familial bullous disease, Narrow-band ultraviolet BAbstract
To evaluate the efficacy and safety of narrow-band ultraviolet B (NB-UVB) in treating refractory Hailey-Hailey Disease (HHD), four HHD patients (36–68 years old, disease duration 5–20 years) were included. NB-UVB was administered twice weekly, with initial doses 0.300–0.500 J/cm2; and 0.050 J/cm2; increments per session, and some patients received concurrent topical medications; follow-up lasted 1.5–3 months, with outcomes assessed via lesion body surface area (BSA) and Visual Analog Scale scores (disease severity, Refractory Visual Analog Scale, pruritus). Onset time was 0.25–4 months: 2 patients achieved complete lesion clearance, the other 2 had 60% and 87.5% BSA reduction, and the longest remission was 18 months; only 1 patient reported moderate irradiation-site pain, with no severe adverse events. NB-UVB shows satisfactory efficacy and good safety in refractory HHD and may serve as a viable treatment option.
Downloads
References
Li H, Chen L, Mei A, Chen L, Xu Y, Hu W, et al. Four novel ATP2C1 mutations in Chinese patients with Hailey-Hailey disease. J Dermatol 2016; 43: 1197–1200. DOI: https://doi.org/10.1111/1346-8138.13407
https://doi.org/10.1111/1346-8138.13407">https://doi.org/10.1111/1346-8138.13407
Hu Z, Bonifas JM, Beech J, Bench G, Shigihara T, Ogawa H, et al. Mutations in ATP2C1, encoding a calcium pump, cause Hailey-Hailey disease. Nat Genet 2000; 24: 61–65. DOI: https://doi.org/10.1038/71701
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 Ca(2+) pump. Hum Mol Genet 2000; 9: 1131–1140. DOI: https://doi.org/10.1093/hmg/9.7.1131
Li X, Zhang D, Ding J, Li L, Wang Z. Identification of ATP2C1 mutations in the patients of Hailey-Hailey disease. BMC Med Genet 2020; 21; 120. DOI: https://doi.org/10.1186/s12881-020-01056-4
LeBlanc KG, Jr, Wharton JB, Sheehan DJ. Refractory Hailey-Hailey disease successfully treated with sandpaper dermabrasion. Skinmed 2011; 9: 263–264
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: 213–215. DOI: https://doi.org/10.3109/14764172.2015.1007063
Lobefaro F, Gualdi G, Andreassi G, Amerio P. Sequential treatments of Hailey-Hailey disease with photodynamic therapy, botulinum toxin type A and dapsone: a case report. Dermatol Ther 2022; 35; e15841. DOI: https://doi.org/10.1111/dth.15841
Hamada T, Umemura H, Aoyama Y, Iwatsuki K. Successful therapeutic use of targeted narrow-band ultraviolet B therapy for refractory Hailey-Hailey disease. Acta Derm Venereol 2013; 93: 110–111. DOI: https://doi.org/10.2340/00015555-1390
Leinonen PT, Hägg PM, Peltonen S, Jouhilahti EM, Melkko J, Korkiamäki T, et al. Reevaluation of the normal epidermal calcium gradient, and analysis of calcium levels and ATP receptors in Hailey-Hailey and Darier epidermis. J Invest Dermatol 2009; 129: 1379–1387. DOI: https://doi.org/10.1038/jid.2008.381
Amar LS, Shabana AH, Oboeuf M, Martin N, Forest N. Involvement of desmoplakin phosphorylation in the regulation of desmosomes by protein kinase C, in HeLa cells. Cell Adhes Commun 1999; 7: 125–138. DOI: https://doi.org/10.3109/15419069909034396
Berth-Jones J, Smith SG, Graham-Brown RA. Benign familial chronic pemphigus (Hailey-Hailey disease) responds to cyclosporin. Clin Exp Dermatol 1995; 20: 70–72. DOI: https://doi.org/10.1111/j.1365-2230.1995.tb01290.x
Vanderbeck KA, Giroux L, Murugan NJ, Karbowski LM. Combined therapeutic use of oral alitretinoin and narrowband ultraviolet-B therapy in the treatment of hailey-hailey disease. Dermatol Rep 2014; 6; 5604. DOI: https://doi.org/10.4081/dr.2014.5604
Mizuno K, Hamada T, Hashimoto T, Okamoto H. Successful treatment with narrow-band UVB therapy for a case of generalized Hailey-Hailey disease with a novel splice-site mutation in ATP2C1 gene. Dermatol Ther 2014; 27: 233–235. DOI: https://doi.org/10.1111/dth.12125
Abaca MC, Flores L, Parra V. Narrowband UV-B phototherapy in the treatment of generalized Hailey-Hailey disease. Actas Dermosifiliogr (Engl Ed) 2018; 109: 924–927. DOI: https://doi.org/10.1016/j.adengl.2018.10.008
Lapa T, Breslavets M. Treatment of Hailey-Hailey disease with narrowband phototherapy and acitretin: a case report. SAGE Open Med Case Rep 2019; 7; 2050313X19845221. DOI: https://doi.org/10.1177/2050313X19845221
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
All digitalized ActaDV contents is available freely online. The Society for Publication of Acta Dermato-Venereologica owns the copyright for all material published until volume 88 (2008) and as from volume 89 (2009) the journal has been published fully Open Access, meaning the authors retain copyright to their work.
Unless otherwise specified, all Open Access articles are published under CC-BY-NC licences, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for non-commercial purposes, provided proper attribution to the original work.
