View of the clinic, diagnosis and treatment of familial benign pemphigus (Hailey — Hailey disease). Literature review

Cover Page


Cite item

Full Text

Abstract

The article describes modern views on predisposing factors, histological and genetic changes, the role of ATP2C1 encoding a mutant gene, localized on chromosome 3 in the pathogenesis of Hailey — Hailey disease. Diagnostic criteria, differential diagnostics with other diseases and methods of modern treatment of this disease are presented.

About the authors

M. V. Drozhdina

Kirov State Medical University

Author for correspondence.
Email: drozhdina@yandex.ru

Cand. Sci. (Med.), Assoc. Prof., Department of Dermatovenerology and Cosmetology

K Marks str., 112, Kirov, 610027, Russian Federation

Russian Federation

S. V. Koshkin

Kirov State Medical University

Email: koshkin_sergei@mail.ru

Dr. Sci. (Med.), Prof., Departmental Head, Department of Dermatovenereology and Cosmetology

K Marks str., 112, Kirov, 610027, Russian Federation

Russian Federation

References

  1. Елькин В. Д., Митрюковский Л. С., Седова Т. Г. Избранная дерматология. Редкие дерматозы и дерматологические синдромы. Иллюстрированный справочник по диагностике и лечению дерматозов. Пермь, 2004. 946 с. + вкл. 197 с.: 537–538. [Elkin V. D., Mitryukovsky L. S., Sedova T. G. Selected dermatology. Rare dermatosis and dermatological syndromes. Illustrated reference book on the diagnosis and treatment of dermatosis. Perm, 2004. 946 p. + incl. 197 p.: 537–538. (In Russ.)]
  2. Nilofar Diwan, Nidhi Jivani, Pragya Nair. Hailey — Hailey disease: 2 case reports. J of Evolution of Med and Dent Sci. 2015;4(63):11083–11089. doi: 10.14260/jemds/2015/1597
  3. Nidhi Yadav, Bhushan Madke, Sumit Kar, Kameshwar Prasad, Nitin Gangane. Hailey — Hailey disease. Indian Dermatol Online J. 2016 Mar-Apr;7(2):147–148. doi: 10.4103/2229-5178.178090
  4. Fairclough R. J., 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):24721–24730.
  5. Foggia L., Aronchik I., Aberg K., Brown B., Hovnanian A., Mauro T. M. 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;119:671–679.
  6. Самцов А. В., Белоусова И. Э. Буллезные дерматозы. Монография. Москва, 2012. 144 с.: 15–16. [Samtsov A. V., Belousov I. E. Bullous dermatoses. Monograph. Moscow, 2012. 144 p. : 15–16. (In Russ.)]
  7. Leinonen P. T., Hagg P. M., 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):1379–1387.
  8. Meng L., Gu Y., Du X. F., Shao M. H., Zhang L. L., Zhang G. L. 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):19349–19359.
  9. Burge S. Hailey — Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol. 1992;126(3):275–282.
  10. Махнева Н. В., Давиденко Е. Б., Черныш Е. С., Белецкая Л. В. Доброкачественная семейная хроническая пузырчатка Гужеро — Хейли — Хейли в аспекте иммунопатологии. Российский журнал кожных и венерических болезней. 2014:(2):32–36. [Makhneva N. V., Davidenko E. B., Chernysh E. S., Beletskaya L. V. Gougerot — Hailey — Hailey’s benign familial chronic pemphigus in the aspect of immunopathology. Russian Journal of Skin and Sexually Transmitted Diseases. 2014:(2):32–36. (In Russ.)]
  11. Leinonen P. T., Hagg P. M., 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):1379–13787.
  12. Lipoff J. B., Mudgil A. V., Young S., Chu P., Cohen S. R. 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):151–154.
  13. Eskin-Schwartz M., Drozhdina M., Sarig O., Koshkin S. et al. Epidermolytic Ichthyosis Sine Epidermolysis. Am J Dermatopathol. 2017;0:1–5.
  14. Кошкин С. В., Чермных Т. В., Евсеева А. Л., Рябова В. В., Рябов А. Н. Ламеллярный врожденный ихтиоз. Вестник дерматологии и венерологии. 2016:(5):44–50. [Koshkin S. V., Chermnykh T. V., Evseeva A. L., Ryabova V. V., Ryabov A. N. Congenital lamellar ichthyosis. Vestnik Dermatologii i Venerologii. 2016:(5):44–50. (In Russ.)]
  15. Villacorta M., Smirnov B. P., Conde J. M., Nousari C. H. Hailey — Hailey Disease Masquerading as Intertriginous Candidiasis for 10 years. J of the American Osteopathic College of Dermatology. 2016 Jun;35:30–33.
  16. Дрождина М. Б., Кошкин С. В. Современный взгляд на клинику, диагностику и лечение вульгарной пузырчатки. Презентация случаев. Иммунопатология, аллергология, инфектология. 2017;(3):6–12. [Drozhdina M. B., Koshkin S. V. A modern view on the clinic, diagnosis and treatment of Pemphigus vulgaris. Presentation of cases. Immunopathology, allergology, infectology. 2017;(3):6–12. (In Russ.)]
  17. Megahed M. Histopathology of Blistering Diseases — With Clinical, Electron Microscopic, Immunological and Molecular Biological Correlations Textbook and Atlas. Berlin, Heidelberg: Springer Verlag, 2004:174–222.
  18. Rabeni E. J., Cunningham N. M. Effective treatment of Hailey — Hailey disease with topical tacrolimus. J Am Acad Dermatol. 2002 Nov;47(5):797–798.
  19. Hunt M. J., Salisbury E. L., Painter D. M., Lee S. Vesiculobullous Hailey — Hailey disease: successful treatment with oral retinoids. Australas J Dermatol. 1996 Nov;37(4):196–198.
  20. Varada S., Ramirez-Fort M.K., Argobi Y., Simkin A.D. Remission of refractory benign familial chronic pemphigus (Hailey — Hailey disease) with the addition of systemic cyclosporine. J Cutan Med Surg. 2015;19(2):163–166.
  21. D’Errico A., Bonciani D., Bonciolini V. et al. Hailey — Hailey disease treated with methotrexate. J Dermatol Case Reports. 2012;6(2):49–51.
  22. Berth-Jones J., Smith S.G., Graham-Brown R.A. Benign familial chronic pemphigus (Hailey — Hailey disease) responds to cyclosporin. Clin Exp Dermatol. 1995;20(1):70–72.
  23. Ormerod A. D., Duncan J., Stankler L. Benign familial pemphigus responsive to cyclosporin, a possible role for cellular immunity in pathogenesis. Br J Dermatol. 1991;124(3):299–300.
  24. Ruiz-Rodriguez R., Alvarez J.G., 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):740–742.
  25. 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):181–184.
  26. Lapiere J. C., Hirsh A., Gordon K. B., Cook B., Montalvo A. Botulinum toxin type A for the treatment of axillary Hailey — Hailey disease. Dermatol Surg. 2000 Apr;26(4):371–374.
  27. Lopez-Ferrer A., Alomar A. Botulinum toxin A for the treatment of familial benign pemphigus. Actas Dermosifiliogr. 2012;103(6):532–535.
  28. Bedi M., Tarylor L. Recalcitrant Hailey — Hailey Disease Responds to Oral Tacrolimus and Botulinum Toxin Type A. Cutis. 2015;96:E14–E16.
  29. Kaniszewska M., Rovner R., Arshanapalli A., Tung R. Oral glycopyrrolate for the treatment of Hailey — Hailey disease. JAMA Dermatol. 2015 Mar 1;151(3):328–329.
  30. Berger E. M., Galadari H. I., Gottlieb A. B. Successful treatment of Hailey — Hailey disease with acitretin. J Drugs Dermatol. 2007 Jul;6(7):734–736.
  31. Hurd D. S., Johnston C., Bevins A. A case report of Hailey — Hailey disease treated with alefacept (Amevive). Br J Dermatol. 2008 Feb;158(2):399–401.
  32. Tang M. B., Tan E. S. Hailey — Hailey disease: effective treatment with topical cadexomer iodine. J Dermatolog Treat. 2011 Oct;22(5):304–305.
  33. 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.
  34. Fisher G. H., Geronemus R. G. Improvement of familial benign pemphigus after treatment with pulsed-dye laser: a case report. Dermatol Surg. 2006 Jul;32(7):966–968.
  35. Fernandez Guarino M., Ryan A.M. et al. Experience with photodynamic therapy in Hailey — Hailey disease. J Dermatolog Treat. 2008;19(5):288–290.

Supplementary files

Supplementary Files
Action
1. JATS XML
Download

Copyright (c) 2018 Drozhdina M.V., Koshkin S.V.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.
СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77 - 60448 от 30.12.2014.


This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies