periodontologyПародонтологияParodontologiya1683-37591726-7269Russian Periodontal Association (RPA)10.33925/1683-3759-2025-1082periodontology-1082Research ArticleКЛИНИЧЕСКИЙ СЛУЧАЙCASE REPORTРубцующий пемфигоид Лорта-Жакоба (проявление в полости рта): клинические случаиCicatricial pemphigoid (Lortat-Jacob disease) with oral manifestations: clinical case descriptionshttps://orcid.org/0000-0002-9980-0969ВолковЕ. А.VolkovE. A.

Волков Евгений Алексеевич, доктор медицинских наук, профессор кафедры терапевтической стоматологии и пародонтологии

Москва

Evgeny A. Volkov, DMD, Professor, Department of the Restorative Dentistry and Periodontology

Moscow

volkov50@inbox.ruhttps://orcid.org/0009-0002-3678-6503ВасюковаО. М.VasyukovaO. M.

Васюкова Ольга Михайловна, кандидат медицинских наук, доцент, ассистент кафедры терапевтической стоматологии и пародонтологии

127006, ул. Долгоруковская, д. 4, г. Москва

Olga M. Vasyukova, DMD, PhD, Docent, Assistant Professor, Department of the Restorative Dentistry and Periodontics

4 Dolgorukovskaya Str., Moscow, 127006

vasyukova7@mail.ruhttps://orcid.org/0000-0002-4527-7471СлажневаЕ. С.SlazhnevaE. S.

Слажнева Екатерина Сергеевна, кандидат медицинских наук, доцент кафедры терапевтической стоматологии и пародонтологии

Москва

Ekaterina S. Slazhneva, DMD, PhD, Associate Professor, Department of the Restorative Dentistry and Periodontology

Moscow

katushkor@mail.ruhttps://orcid.org/0009-0003-5348-3763БазикянО. А.BazikyanO. A.

Базикян Ольга Анатольевна, кандидат медицинских наук, доцент кафедры хирургической стоматологии и имплантологии

Москва

Olga A. Bazikyan, DMD, PhD, Associate Professor, Department of the Oral Surgery and Implantology, Vice-President of RPA

Moscow

prof.bazikian@gmail.comhttps://orcid.org/0000-0002-4141-1370АтрушкевичВ. Г.AtrushkevichV. G.

Атрушкевич Виктория Геннадьевна, доктор медицинских наук, профессор, заведующая кафедрой терапевтической стоматологии и пародонтологии

Москва

Victoria G. Atrushkevich, DMD, PhD, DSc, Professor, Head of the Department of Restorative Dentistry and Periodontology, Vice-President of RPA

Moscow

atrushkevichv@mail.ruРоссийский университет медициныРоссияRussian University of MedicineRussian Federation2025250420253018693Copyright © Волков Е.А., Васюкова О.М., Слажнева Е.С., Базикян О.А., Атрушкевич В.Г., 20252025Волков Е.А., Васюкова О.М., Слажнева Е.С., Базикян О.А., Атрушкевич В.Г.Volkov E.A., Vasyukova O.M., Slazhneva E.S., Bazikyan O.A., Atrushkevich V.G.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.parodont.ru/jour/article/view/1082Актуальность

Актуальность. Рубцующий пемфигоид Лорта-Жакоба (L12.1 по МКБ 10) – редкое хроническое аутоиммунное заболевание, характеризующееся образованием субэпителиальных пузырей и эрозий на поверхности слизистых оболочек с тенденцией к образованию рубцов. С учетом того, что слизистая оболочка рта может явиться местом первого проявления заболевания, возрастает роль стоматолога в комплексной диагностике и лечении указанной патологии.

Описание клинических случаев. В статье представлены два клинических случая проявления рубцующего пемфигоида Лорта-Жакоба. У пациента элементы поражения локализовались на слизистой оболочке рта в виде эрозий на деснах, мягком небе и переходной складке. У пациентки – на мягком и твердом небе, альвеолярном отростке. В связи с поражением глаз пациентам требовалось лечение не только дерматолога, но и офтальмолога.

Заключение

Заключение. Недостаточная изученность данной патологии, многообразие клинических проявлений, возможное развитие тяжелых осложнений требует объединение усилий врачей различных специальностей для ранней диагностики и своевременной терапии заболевания.

Relevance

Relevance. Cicatricial pemphigoid (Lortat-Jacob disease, ICD-10 code L12.1) is a rare chronic autoimmune disorder characterized by the formation of subepithelial blisters and erosions on mucous membranes, typically leading to scarring. Since the oral mucosa may serve as the initial site of manifestation, the dentist plays a critical role in the early identification and comprehensive management of this condition

Clinical case description. This article presents two clinical cases of cicatricial pemphigoid (Lortat-Jacob disease). In the male patient, lesions were localized on the oral mucosa and manifested as erosions on the gingiva, soft palate, and vestibular fold. In the female patient, erosions were observed on the soft and hard palate and the alveolar ridge. Due to ocular involvement, both patients required care not only from a dermatologist but also from an ophthalmologist.

Conclusion

Conclusion. Limited awareness of this condition, the variability of its clinical manifestations, and the potential for severe complications highlight the need for interdisciplinary collaboration to ensure early recognition and timely treatment.

рубцующий пемфигоидаутоиммунное заболеваниепоражение слизистой оболочки полости ртаcicatricial pemphigoidautoimmune diseaseoral mucosal lesionАвторы заявляют об отсутствии внешнего финансирования при проведении исследования.The authors declare that there was no external funding for the study.ReferencesLerner G., Jeremias P., Matthias T. The world incidence and prevalence of autoimmune diseases is increasing. International Journal of Celiac Disease. 2015;3(4):151–155. doi: 10.12691/ijcd-3-4-8Lerner G., Jeremias P., Matthias T. The world incidence and prevalence of autoimmune diseases is increasing. International Journal of Celiac Disease. 2015;3(4):151–155. doi: 10.12691/ijcd-3-4-8Lohi S., Mustalahti K., Kaukinen K., et al. Increasing prevalence of coeliac disease over time. Alimentary Pharmacology & Therapeutics. 2007;26(9):1217–1225. doi: 10.1111/j.1365-2036.2007.03502.x.Lohi S., Mustalahti K., Kaukinen K., et al. Increasing prevalence of coeliac disease over time. Alimentary Pharmacology & Therapeutics. 2007;26(9):1217–1225. doi: 10.1111/j.1365-2036.2007.03502.x.Бутов ЮС, Васенова ВЮ. Буллезные дерматозы. В книге: Бутов ЮС, Скрипкин ЮК, Иванов ОЛ, редакторы. Дерматовенерология. Национальное руководство. Краткое издание. М.: Гэотар-Медиа. 2017:520- 549. Режим доступа: https://elibrary.ru/item.asp?id=54314958Butov YS, Vasenova VY Bullous dermatoses. In: Butov YS, Skripkin YK, Ivanov OL, editors. Dermatovenerology. national guide. brief edition. M.: Geotar-Media. 2017:520-549 (In Russ.). Available from: https://elibrary.ru/item.asp?id=54314958Benoit S, Schmidt E, Sitaru C, Rose C, Goebeler M, Bröcker EB, Zillikens D. Schleimhautpemphigoid mit Autoantikörpern gegen Laminin 5 [Anti-laminin 5 mucous membrane pemphigoid]. J Dtsch Dermatol Ges. 2006;4(1):41-4 (German). doi: 10.1111/j.1610-0387.2005.05826.xBenoit S, Schmidt E, Sitaru C, Rose C, Goebeler M, Bröcker EB, Zillikens D. Schleimhautpemphigoid mit Autoantikörpern gegen Laminin 5 [Anti-laminin 5 mucous membrane pemphigoid]. J Dtsch Dermatol Ges. 2006;4(1):41-4 (German). doi: 10.1111/j.1610-0387.2005.05826.xSchonberg S, Stokkermans TJ. Ocular Pemphigoid. 2023 Jul 31. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. Режим доступа / Available from: https://pubmed.ncbi.nlm.nih.gov/30252356/Schonberg S, Stokkermans TJ. Ocular Pemphigoid. 2023 Jul 31. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. Режим доступа / Available from: https://pubmed.ncbi.nlm.nih.gov/30252356/Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ,, et al. The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch. Dermatol. 2002;38(3):370-379. doi: 10.1001/archderm.138.3.370.Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ,, et al. The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment, and prognostic indicators. Arch. Dermatol. 2002;38(3):370-379. doi: 10.1001/archderm.138.3.370.Kharfi M, Khaled A, Anane R, Fazaa B, Kamoun MR. Early onset childhood cicatricial pemphigoid: a case report and review of the literature. Pediatr Dermatol. 2010;27(2):119-124. doi: 10.1111/j.1525-1470.2009.01079.xKharfi M, Khaled A, Anane R, Fazaa B, Kamoun MR. Early onset childhood cicatricial pemphigoid: a case report and review of the literature. Pediatr Dermatol. 2010;27(2):119-124. doi: 10.1111/j.1525-1470.2009.01079.xMays JW, Sarmadi M, Moutsopoulos NM. Oral manifestations of systemic autoimmune and inflammatory diseases: diagnosis and clinical management. J Evid Based Dent Pract. 2012;12(3 Suppl):265-282. doi: 10.1016/S1532-3382(12)70051-9Mays JW, Sarmadi M, Moutsopoulos NM. Oral manifestations of systemic autoimmune and inflammatory diseases: diagnosis and clinical management. J Evid Based Dent Pract. 2012;12(3 Suppl):265-282. doi: 10.1016/S1532-3382(12)70051-9Tolaymat L, Hall MR. Cicatricial Pemphigoid. 2023 Apr 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. Режим доступа / Available from: https://pubmed.ncbi.nlm.nih.gov/30252376/Tolaymat L, Hall MR. Cicatricial Pemphigoid. 2023 Apr 17. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2025. Режим доступа / Available from: https://pubmed.ncbi.nlm.nih.gov/30252376/Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14(10):930-951. doi: 10.1016/j.autrev.2015.06.005Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev. 2015;14(10):930-951. doi: 10.1016/j.autrev.2015.06.005Магдеева НА, Кобрисева АА, Резникова МА, Мелехина ИФ. Сложности дифференциальной диагностики гранулематоза с полиангиитом и рубцующегося пемфигоида. Архивъ внутренней медицины. 2020;10(5):398-402. doi: 1020514/2226-6704-2020-10-5-398-402Magdeeva NA, Kobriseva AA, Reznikova MA, Melehina IF. Difficulties in the differential diagnosis of granulomatosis with polyangiitis and scarring pemphigoid. The Russian Archives of Internal Medicine. 2020;10(5):398-402 (In Russ.). doi: 1020514/2226-6704-2020-10-5-398-402Rashid H, Lamberts A, Borradori L, Alberti-Violetti S, Barry RJ, Caproni M et al. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology. Part I. J Eur Acad Dermatol Venereol. 2021;35(9):1750-1764. doi: 10.1111/jdv.17397Rashid H, Lamberts A, Borradori L, Alberti-Violetti S, Barry RJ, Caproni M et al. European guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology. Part I. J Eur Acad Dermatol Venereol. 2021;35(9):1750-1764. doi: 10.1111/jdv.17397Shi L, Li X, Qian H. Anti-Laminin 332-Type Mucous Membrane Pemphigoid. Biomolecules. 2022;12(10):1461. doi: 10.3390/biom12101461Shi L, Li X, Qian H. Anti-Laminin 332-Type Mucous Membrane Pemphigoid. Biomolecules. 2022;12(10):1461. doi: 10.3390/biom12101461Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591; quiz 591-4. doi: 10.1067/mjd.2000.107248Fleming TE, Korman NJ. Cicatricial pemphigoid. J Am Acad Dermatol. 2000;43(4):571-591; quiz 591-4. doi: 10.1067/mjd.2000.107248Laskaris G, Sklavounou A, Stratigos J. Bullous pemphigoid, cicatricial pemphigoid, and pemphigus vulgaris. A comparative clinical survey of 278 cases. Oral Surg Oral Med Oral Pathol. 1982;54(6):656-662. doi: 10.1016/0030-4220(82)90080-9Laskaris G, Sklavounou A, Stratigos J. Bullous pemphigoid, cicatricial pemphigoid, and pemphigus vulgaris. A comparative clinical survey of 278 cases. Oral Surg Oral Med Oral Pathol. 1982;54(6):656-662. doi: 10.1016/0030-4220(82)90080-9Vijayan V, Paul A, Babu K, Madhan B. Desquamative gingivitis as only presenting sign of mucous membrane pemphigoid. J Indian Soc Periodontol. 2016;20(3):340-343. doi: 10.4103/0972-124X.182602Vijayan V, Paul A, Babu K, Madhan B. Desquamative gingivitis as only presenting sign of mucous membrane pemphigoid. J Indian Soc Periodontol. 2016;20(3):340-343. doi: 10.4103/0972-124X.182602Mobini N, Nagarwalla N, Ahmed AR. Oral pemphigoid. Subset of cicatricial pemphigoid? Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;85(1):37-43. doi: 10.1016/s1079-2104(98)90395-xMobini N, Nagarwalla N, Ahmed AR. Oral pemphigoid. Subset of cicatricial pemphigoid? Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1998;85(1):37-43. doi: 10.1016/s1079-2104(98)90395-xCizenski JD, Michel P, Watson IT, Frieder J, Wilder EG, Wright JM, et al. Spectrum of orocutaneous disease associations: Immune-mediated conditions. J Am Acad Dermatol. 2017;77(5):795-806. doi: 10.1016/j.jaad.2017.02.019Cizenski JD, Michel P, Watson IT, Frieder J, Wilder EG, Wright JM, et al. Spectrum of orocutaneous disease associations: Immune-mediated conditions. J Am Acad Dermatol. 2017;77(5):795-806. doi: 10.1016/j.jaad.2017.02.019Bertram F, Bröcker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges. 2009;7(5):434-440. doi: 10.1111/j.1610-0387.2008.06976.xBertram F, Bröcker EB, Zillikens D, Schmidt E. Prospective analysis of the incidence of autoimmune bullous disorders in Lower Franconia, Germany. J Dtsch Dermatol Ges. 2009;7(5):434-440. doi: 10.1111/j.1610-0387.2008.06976.xMondino BJ, Linstone FA. Ocular pemphigoid. Clin Dermatol. 1987;5(1):28-35. doi: 10.1016/0738-081x(87)90046-0Mondino BJ, Linstone FA. Ocular pemphigoid. Clin Dermatol. 1987;5(1):28-35. doi: 10.1016/0738-081x(87)90046-0Jascholt I, Lai O, Zillikens D, Kasperkiewicz M. Periodontitis in oral pemphigus and pemphigoid: A systematic review of published studies. J Am Acad Dermatol. 2017;76(5):975-978.e3. doi: 10.1016/j.jaad.2016.10.028Jascholt I, Lai O, Zillikens D, Kasperkiewicz M. Periodontitis in oral pemphigus and pemphigoid: A systematic review of published studies. J Am Acad Dermatol. 2017;76(5):975-978.e3. doi: 10.1016/j.jaad.2016.10.028Mameletzi E, Hamedani M, Majo F, Guex-Crosier Y. Clinical manifestations of mucous membrane pemphigoid in a tertiary center. Klin Monbl Augenheilkd. 2012;229(4):416-419. doi: 10.1055/s-0031-1299403Mameletzi E, Hamedani M, Majo F, Guex-Crosier Y. Clinical manifestations of mucous membrane pemphigoid in a tertiary center. Klin Monbl Augenheilkd. 2012;229(4):416-419. doi: 10.1055/s-0031-1299403Sultan AS, Villa A, Saavedra AP, Treister NS, Woo SB. Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study. Oral Dis. 2017;23(4):498-504. doi: 10.1111/odi.12639Sultan AS, Villa A, Saavedra AP, Treister NS, Woo SB. Oral mucous membrane pemphigoid and pemphigus vulgaris-a retrospective two-center cohort study. Oral Dis. 2017;23(4):498-504. doi: 10.1111/odi.12639Sasaki R, Okamoto T. Autoimmune blisters in the gingiva. BMJ Case Rep. 2020;13(9):e238011. doi: 10.1136/bcr-2020-238011Sasaki R, Okamoto T. Autoimmune blisters in the gingiva. BMJ Case Rep. 2020;13(9):e238011. doi: 10.1136/bcr-2020-238011Labowsky MT, Stinnett SS, Liss J, Daluvoy M, Hall RP 3rd, Shieh C. Clinical Implications of Direct Immunofluorescence Findings in Patients With Ocular Mucous Membrane Pemphigoid. Am J Ophthalmol. 2017;183:48-55. doi: 10.1016/j.ajo.2017.08.009Labowsky MT, Stinnett SS, Liss J, Daluvoy M, Hall RP 3rd, Shieh C. Clinical Implications of Direct Immunofluorescence Findings in Patients With Ocular Mucous Membrane Pemphigoid. Am J Ophthalmol. 2017;183:48-55. doi: 10.1016/j.ajo.2017.08.009Chan LS. Mucous membrane pemphigoid. Clin Dermatol. 2001;19(6):703-711. doi: 10.1016/s0738-081x(00)00196-6Chan LS. Mucous membrane pemphigoid. Clin Dermatol. 2001;19(6):703-711. doi: 10.1016/s0738-081x(00)00196-6Gonzalez-Moles MA, Ruiz-Avila I, Rodriguez-Archilla A, Morales-Garcia P, Mesa-Aguado F, Bascones-Martinez A, et al. Treatment of severe erosive gingival lesions by topical application of clobetasol propionate in custom trays. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95(6):688-692. doi: 10.1067/moe.2003.139Gonzalez-Moles MA, Ruiz-Avila I, Rodriguez-Archilla A, Morales-Garcia P, Mesa-Aguado F, BasconesMartinez A, et al. Treatment of severe erosive gingival lesions by topical application of clobetasol propionate in custom trays. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2003;95(6):688-692. doi: 10.1067/moe.2003.139Assmann T, Becker J, Ruzicka T, Megahed M. Topical tacrolimus for oral cicatricial pemphigoid. Clin Exp Dermatol. 2004;29(6):674-676. doi: 10.1111/j.1365-2230.2004.01619.xAssmann T, Becker J, Ruzicka T, Megahed M. Topical tacrolimus for oral cicatricial pemphigoid. Clin Exp Dermatol. 2004;29(6):674-676. doi: 10.1111/j.1365-2230.2004.01619.xKnudson RM, Kalaaji AN, Bruce AJ. The management of mucous membrane pemphigoid and pemphigus. Dermatol Ther. 2010;23(3):268-280. doi: 10.1111/j.1529-8019.2010.01323.xKnudson RM, Kalaaji AN, Bruce AJ. The management of mucous membrane pemphigoid and pemphigus. Dermatol Ther. 2010;23(3):268-280. doi: 10.1111/j.1529-8019.2010.01323.xStaines K, Hampton PJ. Treatment of mucous membrane pemphigoid with the combination of mycophenolate mofetil, dapsone, and prednisolone: a case series. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(1):e49-56. doi: 10.1016/j.oooo.2012.01.030Staines K, Hampton PJ. Treatment of mucous membrane pemphigoid with the combination of mycophenolate mofetil, dapsone, and prednisolone: a case series. Oral Surg Oral Med Oral Pathol Oral Radiol. 2012;114(1):e49-56. doi: 10.1016/j.oooo.2012.01.030Lytvyn Y, Rahat S, Mufti A, Witol A, Bagit A, Sachdeva M, Yeung J. Biologic treatment outcomes in mucous membrane pemphigoid: A systematic review. J Am Acad Dermatol. 2022;87(1):110-120. doi: 10.1016/j.jaad.2020.12.056Lytvyn Y, Rahat S, Mufti A, Witol A, Bagit A, Sachdeva M, Yeung J. Biologic treatment outcomes in mucous membrane pemphigoid: A systematic review. J Am Acad Dermatol. 2022;87(1):110-120. doi: 10.1016/j.jaad.2020.12.056Lourari S, Herve C, Doffoel-Hantz V, Meyer N, Bulai-Livideanu C, Viraben R, et al. Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients. J Eur Acad Dermatol Venereol. 2011;25(10):1238-1240. doi: 10.1111/j.1468-3083.2010.03889.xLourari S, Herve C, Doffoel-Hantz V, Meyer N, Bulai-Livideanu C, Viraben R, et al. Bullous and mucous membrane pemphigoid show a mixed response to rituximab: experience in seven patients. J Eur Acad Dermatol Venereol. 2011;25(10):1238-1240. doi: 10.1111/j.1468-3083.2010.03889.xГорячева ТП, Островская ЮВ, Алешина ОА, Давыдова СИ, Горячева ИД. Применение аутофлуоресцентной стоматоскопии в алгоритме диагностики патологических состояний слизистой оболочки рта и красной каймы губ у подростков. Стоматология детского возраста и профилактика. 2024;24(3):267-275. doi:10.33925/1683-3031-2024-836Goryacheva TP, Ostrovskaya YuV, Aleshina OA, Davydova SI, Goryacheva ID. Use of autofluorescent stomatoscopy in the diagnostic algorithm for oral mucosal and vermilion border lesions in adolescents. Pediatric dentistry and dental prophylaxis. 2024;24(3):267-275 (In Russ.). doi:10.33925/1683-3031-2024-836Sacher C, Hunzelmann N. Cicatricial pemphigoid (mucous membrane pemphigoid): current and emerging therapeutic approaches. Am J Clin Dermatol. 2005;6(2):93-103. doi: 10.2165/00128071-200506020-00004Sacher C, Hunzelmann N. Cicatricial pemphigoid (mucous membrane pemphigoid): current and emerging therapeutic approaches. Am J Clin Dermatol. 2005;6(2):93-103. doi: 10.2165/00128071-200506020-00004Тарасенко ГН, Гладько ВВ, Сысоева АН, Свидерская ОС, Кузьмина. ЮВ, Токмакова АН. Рубцующийся пемфигоид: междисциплинарная проблема. Российский журнал кожных и венерических болезней. 2017.20(3):154-156. doi: 10.18821/1560-9588-2017-20-3-154-156Tarasenko GN, Glalko VV, Sysoeva AN, Sviderskaya OS, KuzminaYuV, Tokmakova AN. Cicatricial pemphigoid: an interdisciplinary problem. Russian Journal of Skin and Venereal Diseases. 201.20(3):154-156 (In Russ). doi: 10.18821/1560-9588-2017-20-3-154-156

The authors declare that there are no conflicts of interest present.