Current methods of treatment of true acantholytic pemphigus

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Abstract

Major stages of the pathogenesis of true acantholytic pemphigus are interpreted in terms of autoimmune pathology; therefore, treatment of this group of diseases remains pathogenetic and is aimed at suppressing the synthesis of autoantibodies against keratinocyte proteins. Treatment with the use of systemic glucocorticosteroids is currently the main method of treatment for true acantholytic pemphigus. To reduce the course dose of glucocorticosteroids, decrease the risk of adverse effect development and achieve long-term remission, further studies of disease pathogenesis and development of new treatment methods for reducing the doses of glucocorticosteroids are of great importance.

About the authors

A. A. Kubanov

State Research Center of Dermatovenereology and Cosmetology, Ministry of healthcare of the Russian Federation; Russian Medical Academy of Postgraduate Studies, Ministry of Health of the Russian Federation

Author for correspondence.
Email: noemail@neicon.ru
Россия

T. V. Abramova

Russian Medical Academy of Postgraduate Studies, Ministry of Health of the Russian Federation

Email: abtava@mail.ru
Россия

References

  1. Torsuev N.A., Sheklakov N.D.Romanenko V.N. Bulleznye dermatozy. M: Meditsina. 1979; 296. [Торсуев Н.А., Шеклаков Н.Д. Романенко В. Н. Буллезные дерматозы. М: Медицина. 1979; 296.]
  2. Schiltz J.R., Michel B. Production of epidermal acantholysis in normal humanskin in vitro by the IgG fraction from pemphigus serum. J Invest Dermatol 1976; 67 (2): 254-260.
  3. Matushevskaya E.V., Svirshchevskaya E.V., Kubanova A.A. et al. Immunologiya vul'garnoy puzyrchatki i vozmozhnyy mekhanizm formirovaniya zabolevaniya, Vestn Dermatol Venerol 1996; 2: 25-28. [Матушевская Е.В., Свирщевская Е.В., Кубанова А.А. и др. Иммунология вульгарной пузырчатки и возможный механизм формирования заболевания. Вестн дерматол и венерол 1996; 2: 25-28.] Литература
  4. Stanley J.R., Yaar M., Hawley-Nelson P., Katz S.I. Pemphigus antibodies identify a cell surface glycoprotein synthesized by human and mouse keratinocytes. J Clin Invest 1982 Aug; 70 (2): 281-288.
  5. Grando S.A. Pemphigus autoimmunity: hypotheses and realities. Autoimmunity 2012; 45: 1: 7-35.
  6. Kalantari-Dehaghi M., Anhalt G.J., Camilleri M.J. Pemphigus vulgaris autoantibody profiling by proteomic technique. PLoS One 2013; 8 (3): 577-587.
  7. Tsunoda K., Ota T., Aoki M. Induction of pemphigus phenotype by a mouse monoclonal antibody against the amino-terminal adhesive interface of desmoglein 3. J Immunol 2003; 15, 170 (4): 2170-2178.
  8. Grando S.A., Bystryn J.C., Chernyavsky A.I. et al. Apoptolysis: A novel mechanism of skin blistering in pemphigus vulgaris linking the apoptotic pathways to basal cell shrinkage and suprabasal acantholysis. Exp Dermatol. 2009; 18: 764-770.
  9. Sokolovskiy E.V. Puzyrnye dermatozy. Psoriaz. Sovremennye metody lecheniya. Seriya «Biblioteka vracha-dermtaovenerologa». Vypusk 3: SPb: SOTIS. 1999; 134. [Соколовский Е.В. Пузырные дерматозы. Псориаз. Современные методы лечения. Серия «Библиотека врача-дерматовенеролога». Выпуск 3: СПб: СОТИС. 1999; 134.]
  10. Kalamkaryan A.A., Trofimova L.Ya., Khapilova V.I. Immunodepressanty v kompleksnoy terapii puzyrchatki. Vestn Dermatol Venerol 1979; 12: 28-32. [Каламкарян А.А., Трофимова Л.Я., Хапилова В.И. Иммунодепрессанты в комплексной терапии пузырчатки. Вестн дерматол и венерол 1979; l2: 28-32.]
  11. Dermatovenerologiya, 2010. (Klinicheskie rekomendatsii / Rossiyskoe obshchestvo dermatovenerologov) / [pod red. A. A. Kubanovoy]. M.: DEKS-PRESS, 2010; 428. [Дерматовенерология, 2010.(Клинические рекомендации Российское общество дерматовенерологов [под ред. А.А. Кубановой]. М: ДЭКС-ПРЕСС, 2010; 428.]
  12. Mao X., Payne A.S. Seeking approval: Present and future therapies for pemphigus vulgaris. Curr Opin Investig Drugs May 2008; 9 (5): 497-504.
  13. Thorn G. W., Forsham P. Y., Frawley T. F. et al. The clinical usefulness of ACTH and cortisone. New Engl J Med 1950; 242: 20.
  14. Mainali E.S., Kikuchi T., Tew J.G. Dexamethasone inhibits maturation and alters function of monocyte-derived dendritic cells from cord blood. Pediatr Res 2005; 58: 125-131.
  15. Udey MC, Stanley JR: Pemphigus: Diseases of anti-desmosomal autoimmunity. JAMA 282: 572, 1999.
  16. DeKruyff R.H, Fang Y., Umetsu D.T. Corticosteroids enhance the capacity of macrophages to induce Th2 cytokine synthesis in CD4 Jj lymphocytes by inhibiting IL-12 production. J Immunol 1998; 160: 2231-2237.
  17. Chryssomallis F., Dimitriades A., Chaidemenos G.C. et al. Steroid-pulse therapy in pemphigus vulgaris long term follow-up. Int J Dermatol 1995; 34: 438-442.
  18. Nguyen V.T., Arredondo J., Chernyavsky A.I. et al. Pemphigus vulgaris IgG and methylprednisolone exhibit reciprocal effects on keratinocytes. J Biol Chem 2004; 279: 2135-2146.
  19. Samtsov A.V., Belousova I.E. Bulleznye dermatozy: Monografiya. SPb.: OOO «Izdatel'sko-poligraficheskaya kompaniya «Kosta». 2012: 144. [Самцов А.В., Белоусова И.Э. Буллезные дерматозы: Монография. СПб.: ООО «Издательско-полиграфическая компания «Коста». 2012: 144.]
  20. Mashkilleyson A.L., Antonova T.N., Glebova L.I. et al. Kombinirovannaya terapiya pemfigusa kortikosteroidami i metotreksatom. Vestn Dermatol Venerol 1977; 3: 60-63. [Машкиллейсон А.Л., Антонова Т.Н., Глебова Л.И. и др. Комбинированная терапия пемфигуса кортикостероидами и метотрексатом. Вестн дерматол и венерол 1977; 3: 60-63.]
  21. Mashkilleyson A.L., Kutin S.A., Abramova E.I. i dr. Kozhnye i venericheskie bolezni. Pod red. A.L. Mashkilleysona. M: Meditsina, 1986: 256. [Машкиллейсон А.Л., Кутин С.А., Абрамова Е.И. и др. Кожные и венерические болезни. Под ред. А. Л. Машкиллейсона. М: Медицина 1986: 256.]
  22. Potekaev N.S., Sergeev Yu.V., Yastrebova R.I. i soavt. Skhema snizheniya nizkikh doz kortikosteroidnykh gormonov pri istinnoy puzyrchatke. Klin dermatol venerol. 2003; 3: 36-40. [Потекаев Н.С., Сергеев Ю.В. Ястребова Р.И. и соавт. Схема снижения низких доз кортикостероидных гормонов при истинной пузырчатке. Клин дерматол и венерол 2003; 3: 36-40.]
  23. Schmidt E., Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Ärztebl Int 2011; 108: 399-405.
  24. Tsurova Z.S., Svirshchevskaya E.V., Viskova N.Yu. i dr. Kliniko-immunologicheskiy analiz primeneniya diprospana dlya lecheniya vul'garnoy puzyrchatki. Vestn dermatovenerol. 1997; 5: 4-7. [Цурова З.С., Свирщевская Е.В., Вискова Н.Ю. и др. Клинико-иммунологический анализ применения дипро-спана для лечения вульгарной пузырчатки. Вестн дерматовенерол. 1997; 5: 4-7.]
  25. Leventhal J.S., Sanchez M.R. Is it time to reevaluate the treatment of pemphigus? J Drugs Dermatol 2012; 11 (10): 1200-1206.
  26. Ruocco E., Wolf R., Ruocco V. et al. Pemphigus: Associations and management guidelines: Facts and controversies. Clin Dermatol. 2013; 31 (4): 382-390.
  27. Reshetnikova T.B., Lykova S.G., Spitsyna A.V., Makarova Ya. Yu. Vliyanie gormonal'noy terapii na sostoyanie immunnoy sistemy, chastotu i kharakter oslozhneniy u bol'nykh istinnoy akantoliticheskoy puzyrchatkoy v zavisimosti ot iskhodnogo sostoyaniya T-khelperov. Sib Med J 2004; 4: 13-17. [Решетникова Т.Б. Лыкова С.Г., Спицына А.В., Макарова Я.Ю. Влияние гормональной терапии на состояние иммунной системы, частоту и характер осложнений у больных истинной акантолитической пузырчаткой в зависимости от исходного состояния Т-хелперов. Сиб мед журн 2004; 4: 13-17.]
  28. Mimouni D., Bar H., Gdalevich M. et al. Pemphigus, analysis o 155 patients. - J. Eur. Dermatol. Venerol. 2010; 24 (8): 947-952.
  29. Uçmak D, Harman M, Uçmak F, Akpolat V. The frequency of osteoporosis in patients with pemphigus vulgaris on treatment // Indian J Dermat Venereol Leprol 2013; 79, 2: 211-215.
  30. Olszewska M., Kolacinska-Strasz Z. Sulej J. Efficacy and safety of cyclophosphamide, azathioprine, and cyclosporine (ciclosporin) as adjuvant drugs in pemphigus vulgaris. Am J Clin Dermatol. 2007; 8 (2): 85-92.
  31. Cronstein B. How does methotrexate suppress inflammation? Clin. Exp. Rheumatol. 2010; 28 (5 Suppl. 61): 21-23.
  32. Sterry W., Griffiths C., Korting H.C. EDF Guidelines for Dermatology in Europe 2009, paperback, 376.
  33. Meggitt S.J., Anstey A.V., Mohd Mustapa M.F. et al. British Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine. Br. J. Dermatol. 2011; 165 (4): 711-734.
  34. Allison A.C. Mechanisms of action of mycophenolate mofetil. Lupus. 2005; 14: 2-8.
  35. Halloran P.F. Molecular mechanisms of new immunosuppressants. Clin Transplant 1996; 10 (1 Pt 2): 118-123.
  36. Enk A., Knop J. Mycophenolate is effective in the treatment of pemphigus. Arch Dermatol 1999; 135 (1): 54-56.
  37. Medved A., Maxwell I. Intermittent cyclophosphamide in pemphigus vulgaris and bullous pemphigoid. Can Med Assoc J 1974; 111: 245-246.
  38. Hausheer F.H., Singh U.C., Saxe J.D. et al. Identification of local determinants of DNA interstrand crosslink formation by cyclophosphamide metabolites. Anticancer Drug Des 1989; 4 (4): 281-294.
  39. Eming R., Rech J., Barth S. et al. Prolonged clinical remission of patients with severe pemphigus upon rapid removal of desmoglein-reactive autoantibodies by immunoadsorption. Dermatology 2006; 212 (2): 177-187.
  40. Bystryn J.C. Adjuvant therapy of pemphigus. Arch Dermatol 1984; 120 (7): 941-951.
  41. Sharma V.K., Khandpur S. Evaluation of cyclophosphamide pulse therapy as an adjuvant to oral corticosteroid in the management of pemphigus vulgaris. Clin Exp Dermatol 2013; 38 (6): 659-664.
  42. Kubanova A.A., Samsonov V.A., Khapilova V.I., Matushevskaya E. V. Sandimmun v terapii bol'nykh istinnoy puzyrchatkoy. Vestn Dermatol Venerol 1994; 6: 42. [Кубанова А.А., Самсонов В.А., Хапилова В.И., Матушевская Е.В. Сандиммун в терапии больных истинной пузырчаткой. Вестн дерматол и венерол 1994; 6: 42.]
  43. Matushevskaya E.V., Kubanova A.A., Samsonov V.A. i dr. Autoantitela i autoantigeny pri puzyrchatke i pemfigoide. Vestn Dermatol Venerol. 1995; 5: 28-33. [Матушевская Е.В., Кубанова А.А., Самсонов В.А. и др. Аутоантитела и аутоантигены при пузырчатке и пемфигоиде. Вестн дерматол и венерол 1995; 5: 28-33.]
  44. Bystryn J. C., Jiao D. IVIg selectively and rapidly decreases circulating pathogenic autoantibodies in pemphigus vulgaris. Autoimmunity 2006; 39 (7): 601-607.
  45. Amagai M., Ikeda S., Shimizu H. et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol 2009; 60 (4): 595-603.
  46. Gürcan H.M., Jeph S., Ahmed A.R. Intravenous immunoglobulin therapy in autoimmune mucocutaneous blistering diseases: a review of the evidence for its efficacy and safety. Am J Clin Dermatol 2010; 11 (5): 315-326.
  47. Ahmed A.R., Gurcan H.M. Use of intravenous immunoglobulin therapy during pregnancy in patients with pemphigus vulgaris. J Eur Acad Dermatol Venerol 2011; 25: 1073-1079.
  48. Browning J.L. B cells move to centre stage: novel opportunities for autoimmune disease treatment. Nat Rev Drug Discov 2006; 5: 564-576.
  49. Joly P., Monquet H., Rujeau J.C. et al. A single cycle of rituximab for the treatment of severe pemphigus. New Engl J Med 2007; 357: 545-552.
  50. Craythorne E.E., Mufti G., DuVivier A.W. Rituximab used as a first-line single agent in the treatment of pemphigus vulgaris. J Am Acad Dermatol 2011; 65: 1064-1065.
  51. Kanwar A.J., Vinay K. Rituximab in pemphigus. Indian J Dermatol Venereol Leprol 2012; 78 (6): 671-676.
  52. Cianchini G., Lupi F., Masini C. еt al. Therapy with rituximab for autoimmune pemphigus: results from a single-center observational study on 42 cases with long-term follow-up. J Am Acad Dermatol 2012; 67 (4): 617-622.
  53. Horvath B., Huizinga J., Pas H. H. et al. Low-dose rituximab is effective in pemphigus. Br J Dermatol 2012; 166 (2): 405-412.
  54. Pardo J., Mercader P., Mahiques L. et al. Infliximab in the management of severe pemphigus vulgaris. Br J Dermatol. 2005; 153 (1): 222-223.
  55. Garcia-Rabasco A., Alsina-Gibert M., PauCharles I. et al. Infliximab therapy failure in two patients with pemphigus vulgaris. J Am Acad Dermatol 2012; 67 (5): 196-197.
  56. Shetty A., Marcum C.B., Glass L.F., Carter J.D. Successful treatment of pemphigus vulgaris with etanercept in four patients. J Drugs Dermatol 2009; 8 (10): 940-943.
  57. Fiorentino D.F., Garcia M.S., Rehmus W. et al. A pilot study of etanercept treatment for pemphigus vulgaris. Arch Dermatol 2011; 147 (1): 117-118.
  58. Howell S.M., Bessinger G.T., Altman C.E. et al. Rapid response of IgA pemphigus of the subcorneal pustular dermatosis subtype to treatment with adalimumab and mycophenolate mofetil. J Am Acad Dermatol 2005; 53: 541-543.
  59. Vojâckovâ N., Fialova J., Vanousovâ D., Herco-govâ J. et al. Pemphigus vulgaris treated with adalimumab: case study. Dermatol Ther 2012; 25: 95-97.
  60. Angelini G., Bonamonte D., Lucchese A., Preliminary data on pemphigus vulgaris treatment by a proteomics-defined peptide: a case report. J Transl Med 2006; 4: 43.
  61. Perez O.A., Patton T.Novel therapies for pemphigus vulgaris: an overview.Drugs Aging 2009; 26 (10): 833-846.
  62. Koga H., Tsuruta D., Ohyama B.Desmoglein 3, its pathogenecity and a possibility for therapeutic target in pemphigus vulgaris. Expert Opin Ther Targets 2013; 17 (3): 293-306.
  63. Grando S.A., Glukhen'kiy B.T., Romanenko A.B. et al. Mekhanismy terapevticheskogo deystviya ekstrakorporal'noy detoksikatsii pri autoimmunnykh bulleznykh dermatozakh. Vestn dermatol venerol 1988; 7: 6-11. [Грандо С.А., Глухенький Б.Т., Романенко А.Б. и др. Механизмы терапевтического действия экстракорпоральной детоксикации при аутоиммунных буллезных дерматозах. Вестн дерматол и венерол 1988; 7: 6-11.]
  64. Kil'dyushevskiy A.V., Molochkov V.A., Karzanov O.V. Dinamika kletochnogo immuniteta v protsesse ekstrakorporal'noy fotokhimioterapii u bol'nykh istinnoy puzyrchatkoy. Rus J Skin Sex Transmit Dis 2008; 4: 71-76. [Кильдюшевский А.В., Молочков В.А., Карзанов О.В. Динамика клеточного иммунитета в процессе экстракорпоральной фотохимиотерапии у больных истинной пузырчаткой. Росс журн кожн вен бол 2008; 4: 71-76.]
  65. Yamada H., Yamaguchi H., Takamori K. et al. Plasmapheresis for the treatment of pemphigus and bullous pemphigoid. Ther Apher 1997; 1: 178-82.
  66. Eming R., Hertl M. Immunoadsorption in pemphigus. Autoimmunity. 2006; 39 (7): 609-616.
  67. Meyersburg D., Schmidt E., Kasperkiewicz M., Zillikens D. Immunoadsorption in Dermatology. Ther Apher Dial 2012; 16 (4): 311-320.
  68. Grando S.A., Glukhen'kaya A.B., Ivanchenko L.L. Vliyanie enterosorbtsii na mikrotsirkulyatsiyu i klinicheskuyu kartinu pri zudyashchikh dermatozakh. Vestn Dermatol Venerol 1987; 2: 41-44. [Грандо С.А., Глухенькая А.Б., Иванченко Л.Л. Влияние энтеросорбции на микроциркуляцию и клиническую картину при зудящих дерматозах. Вестн дерматол и венерол 1987; 2: 41-44.]
  69. Bakos L., Zoratto G., Brunetto L. et al. Photodynamic therapy: a useful adjunct therapy for recalcitrant ulceration in pemphigus vulgaris. J Eur Acad Dermatol Venereol 2009; 23 (5): 599-600.
  70. Hawayek L., Mutasim D. Pemphigus vulgaris. xPharm Online Pharmacology Reference and Database. Elsevier Science, 2004. http: //www. xpharm. com.
  71. Nagasaka T., Fujii Y., Ishida A. et al. Evaluating efficacy of plasmapheresis for patients with pemphigus using desmoglein enzyme-linked immunosorbent assay. Br J Dermatol 2008; 158 (4): 685-690.
  72. Roujeau J. C. Plasmapheresis therapy of pemphigus and bullous pemphigoid. Semin Dermatol 1988; 7 (3): 195-200.
  73. Grebennikov V.A., Belyavskiy A.D., Kaminskiy M.Yu. Izuchenie immunokorrigiruyushchego i detoksitsiruyushchego vozdeystviya gemosorbtsii, plazmafereza i enterosorbtsii pri puzyrchatke. Vestn Dermatol Venerol 1990; 5: 33-38. [Гребенников В.А., Белявский А.Д., Каминский М.Ю. Изучение иммунокорригирующего и детоксицирующего воздействия гемосорбции, плазмафереза и энтеросорбции при пузырчатке. Вестн дерматол и венерол 1990; 5: 33-38.]
  74. Schmidt E., Klinker E., Opitz A. et al. Protein A immunoadsorption: a novel and effective adjuvant treatment of severe pemphigus. Br J Dermatol 2003; 148: 1222-1229.
  75. Shimanovich I., Nitschke M., Rose C. et al. Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins. Br J Dermatol 2008; 158: 382-388.
  76. Behzad M., Möbs C., Kneisel A. et al. Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult-to-treat pemphigus vulgaris. Br J Dermatol 2012; 166 (1): 844-852.
  77. Sondergaard K., Carstens J., Jorgensen J. The steroid-sparing effect of long-term plasmapheresis in pemphigus. Acta Dermatol Venerol 1995; 75: 2. 150-152.
  78. Shimanovich I., Herzog S., Schmidt E. et al. Improved protocol for treatment of pemphigus vulgaris with protein A immunoadsorption. Clin Exp Dermatol 2006; 31 (6): 768-774.
  79. Marker M., Derfler K., Monshi B, Rappersberger K. Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis. J Dtsch Dermatol Ges 2011; 19 (1): 27-31.
  80. Luftl M., Stauber A., Mainka A. et al. Successful removal of pathogenic autoantibodies in pemphigus by immunoadsorption with a tryptophan-linked polyvinylalcohol adsorber. Br J Dermatol 2003; 149: 598-605.

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