Familial Benign Pemphigus (Hailey-Hailey Disease): Background, Pathophysiology, Etiology (2024)

  1. Hailey H, Hailey H. Familial benign chronic pemphigus. Arch Dermatol. 1939. 39:679-85.

  2. Fairclough RJ, Dode L, Vanoevelen J, et al. Effect of Hailey-Hailey Disease mutations on the function of a new variant of human secretory pathway Ca2+/Mn2+-ATPase (hSPCA1). J Biol Chem. 2003 Jul 4. 278(27):24721-30. [QxMD MEDLINE Link].

  3. Foggia L, Aronchik I, Aberg K, Brown B, Hovnanian A, Mauro TM. Activity of the hSPCA1 Golgi Ca2+ pump is essential for Ca2+-mediated Ca2+ response and cell viability in Darier disease. J Cell Sci. 2006 Feb 15. 119:671-9. [QxMD MEDLINE Link].

  4. Leinonen PT, Hagg PM, Peltonen S, 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 Jun. 129(6):1379-87. [QxMD MEDLINE Link].

  5. Meng L, Gu Y, Du XF, Shao MH, Zhang LL, Zhang GL, et al. Two novel ATP2C1 mutations in patients with Hailey-Hailey disease and a literature review of sequence variants reported in the Chinese population. Genet Mol Res. 2015 Dec 29. 14 (4):19349-59. [QxMD MEDLINE Link].

  6. Zhu YG, Yang S, Gao M, et al. Two novel mutations of the ATP2C1 gene in Chinese families with Hailey-Hailey disease. J Dermatol Sci. 2006 May. 42(2):125-7. [QxMD MEDLINE Link].

  7. Lipoff JB, Mudgil AV, Young S, Chu P, Cohen SR. Acantholytic dermatosis of the crural folds with ATP2C1 mutation is a possible variant of Hailey-Hailey Disease. J Cutan Med Surg. 2009 May-Jun. 13(3):151-4. [QxMD MEDLINE Link].

  8. Zhang D, Huo J, Li R, Zhang Y, Wang Z, Li X. Altered levels of focal adhesion and extracellular matrix-receptor interacting proteins were identified in Hailey-Hailey disease by quantitative iTRAQ proteome analysis. J Cell Biochem. 2019 Mar. 120 (3):3801-3812. [QxMD MEDLINE Link].

  9. Hunt MJ, Salisbury EL, Painter DM, Lee S. Vesiculobullous Hailey-Hailey disease: successful treatment with oral retinoids. Australas J Dermatol. 1996 Nov. 37(4):196-8. [QxMD MEDLINE Link].

  10. Haley CT, Mui UN, Tyring SK. Safety of Topical Calcineurin Inhibitors for Hailey-Hailey Disease-Reply. JAMA. 2018 Sep 18. 320 (11):1200-1201. [QxMD MEDLINE Link].

  11. Rabeni EJ, Cunningham NM. Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol. 2002 Nov. 47(5):797-8. [QxMD MEDLINE Link].

  12. Ruiz-Rodriguez R, Alvarez JG, Jaen P, Acevedo A, Cordoba S. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). J Am Acad Dermatol. 2002 Nov. 47(5):740-2. [QxMD MEDLINE Link].

  13. Konrad H, Karamfilov T, Wollina U. Intracutaneous botulinum toxin A versus ablative therapy of Hailey-Hailey disease--a case report. J Cosmet Laser Ther. 2001 Dec. 3(4):181-4. [QxMD MEDLINE Link].

  14. Lapiere JC, Hirsh A, Gordon KB, Cook B, Montalvo A. Botulinum toxin type A for the treatment of axillary Hailey-Hailey disease. Dermatol Surg. 2000 Apr. 26(4):371-4. [QxMD MEDLINE Link].

  15. Lopez-Ferrer A, Alomar A. Botulinum toxin A for the treatment of familial benign pemphigus. Actas Dermosifiliogr. 2012 Aug 8.

  16. Kaniszewska M, Rovner R, Arshanapalli A, Tung R. Oral glycopyrrolate for the treatment of hailey-hailey disease. JAMA Dermatol. 2015 Mar 1. 151(3):328-9. [QxMD MEDLINE Link].

  17. Berger EM, Galadari HI, Gottlieb AB. Successful treatment of Hailey-Hailey disease with acitretin. J Drugs Dermatol. 2007 Jul. 6(7):734-6. [QxMD MEDLINE Link].

  18. Hurd DS, Johnston C, Bevins A. A case report of Hailey-Hailey disease treated with alefacept (Amevive). Br J Dermatol. 2008 Feb. 158(2):399-401. [QxMD MEDLINE Link].

  19. Tang MB, Tan ES. Hailey-Hailey disease: effective treatment with topical cadexomer iodine. J Dermatolog Treat. 2011 Oct. 22(5):304-5. [QxMD MEDLINE Link].

  20. 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. 2012 Jun 27.

  21. Awadalla F, Rosenbach A. Effective treatment of hailey-hailey disease with a long-pulsed (5 ms) alexandrite laser. J Cosmet Laser Ther. 2011/08. 13(4):191-2.

  22. Pagliarello C, Paradisi A, Dianzani C, Paradisi M, Persichetti P. Topical tacrolimus and 50% zinc oxide paste for Hailey-Hailey disease: less is more. Acta Derm Venereol. 2012 Jul. 92(4):437-8. [QxMD MEDLINE Link].

  23. Monica Bedi and Lowery Tarylor. Recalcitrant Hailey-Hailey Disease Responds to Oral Tacrolimus and Botulinum Toxin Type A. Cutis. 2015. 96:E14-E16.

  24. Don PC, Carney PS, Lynch WS, Zaim MT, Hassan MO. Carbon dioxide laserabrasion: a new approach to management of familial benign chronic pemphigus (Hailey-Hailey disease). J Dermatol Surg Oncol. 1987 Nov. 13(11):1187-94. [QxMD MEDLINE Link].

  25. Fisher GH, Geronemus RG. Improvement of familial benign pemphigus after treatment with pulsed-dye laser: a case report. Dermatol Surg. 2006 Jul. 32(7):966-8. [QxMD MEDLINE Link].

  26. Kartamaa M, Reitamo S. Familial benign chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser vaporization. Arch Dermatol. 1992 May. 128(5):646-8. [QxMD MEDLINE Link].

  27. Fernandez Guarino M, Ryan AM, et al. Experience with photodynamic therapy in Hailey-Hailey disease. J Dermatolog Treat. 2008. 19(5):288-90. [QxMD MEDLINE Link].

  28. Fisher BK, Margesson LJ. Hailey-Hailey disease (familial benign chronic pemphigus). Genital Skin Disorders: Diagnosis and Treatment. St. Louis, Mo: Mosby-Year Book; 1998. 68, 117.

  29. Yoto A, Makino T, Mizawa M, Matsui Y, Takemoto K, Furukawa F, et al. Two cases of Hailey-Hailey disease effectively treated with apremilast and a review of reported cases. J Dermatol. 2021 Dec. 48 (12):1945-1948. [QxMD MEDLINE Link].

  30. Alajmi A, Jfri A, Lovett A. Hailey-Hailey disease treated successfully with naltrexone and magnesium. JAAD Case Rep. 2019 Sep. 5 (9):760-762. [QxMD MEDLINE Link].

Familial Benign Pemphigus (Hailey-Hailey Disease): Background, Pathophysiology, Etiology (2024)
Top Articles
Latest Posts
Article information

Author: Amb. Frankie Simonis

Last Updated:

Views: 5377

Rating: 4.6 / 5 (76 voted)

Reviews: 91% of readers found this page helpful

Author information

Name: Amb. Frankie Simonis

Birthday: 1998-02-19

Address: 64841 Delmar Isle, North Wiley, OR 74073

Phone: +17844167847676

Job: Forward IT Agent

Hobby: LARPing, Kitesurfing, Sewing, Digital arts, Sand art, Gardening, Dance

Introduction: My name is Amb. Frankie Simonis, I am a hilarious, enchanting, energetic, cooperative, innocent, cute, joyous person who loves writing and wants to share my knowledge and understanding with you.