Mucous Membrane Pemphigoid with Floor of Mouth Involvement: Case Report
Year 2025,
Volume: 11 Issue: 2, 109 - 114, 29.08.2025
Müberra Deniz
,
Can Aydın
,
Tuğrul Kırtıloğlu
,
Abbas Akbari
Abstract
ABSTRACT:
Objective:
Mucous membrane pemphigoid (MMP) is a rare autoimmune disease characterized by subepithelial bullae on mucosal surfaces. The aim of this case report is to highlight the involvement of the floor of the mouth—a localization rarely reported in the literature—and the therapeutic approach for lesions in this region.
Materials and Methods:
A 59-year-old female patient presented to our clinic with complaints of gingival bleeding and periodic bullae. Clinical examination revealed extensive oral involvement, including the floor of the mouth. Following a thorough clinical evaluation and histopathological analysis, a diagnosis of MMP was established. The patient was treated with a systemic regimen consisting of methylprednisolone, dapsone, and mycophenolate mofetil.
Results:
During the six-month follow-up period, a marked regression of the oral lesions and clinical improvement were observed. The patient responded favorably to the treatment.
Conclusion:
This case is significant as it demonstrates that MMP can also involve the floor of the mouth. Early diagnosis and appropriate systemic therapy are crucial for achieving successful outcomes.
References
-
1. 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;138:370–
379.
-
2. Ahmed AR, Hombal SM. Cicatricial pemphigoid. Int J
Dermatol. 1986;25:90–96.
-
3. Bagan J, Muzio LL, Scully C. Number III mucous
membrane pemphigoid. Oral Dis. 2005;11:197–218.
-
4. Hofmann SC, Günther C, Böckle BC, Didona D,
Ehrchen J, Gaskins M, et al. S2k guideline for the
diagnosis and treatment of mucous membrane
pemphigoid. J Dtsch Dermatol Ges. 2022;20:1530–1550.
-
5. Du G, Patzelt S, van Beek N, Schmidt E. Mucous
membrane pemphigoid. Autoimmun Rev.
2022;21:103036.
-
6. Srikumaran D, Akpek EK. Mucous membrane
pemphigoid: recent advances. Curr Opin Ophthalmol.
2012;23:523–527.
-
7. Leao J, Ingafou M, Khan A, Scully C, Porter S.
Desquamative gingivitis: retrospective analysis of
disease associations of a large cohort. Oral Dis.
2008;14:556–560.
-
8. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet.
2013;381:320–332.
-
9. Scott JE, Ahmed AR. The blistering diseases. Med
Clin North Am. 1998;82:1239–1283.
-
10. Parker SR, MacKelfresh J. Autoimmune blistering
diseases in the elderly. Clin Dermatol. 2011;29:69–79.
-
11. Broussard KC, Leung TG, Moradi A, Thorne JE,
Fine J-D. Autoimmune bullous diseases with skin and
eye involvement: cicatricial pemphigoid, pemphigus
vulgaris, and pemphigus paraneoplastica. Clin Dermatol.
2016;34:205–213.
-
12. Ciarrocca KN, Greenberg MS. A retrospective study
of the management of oral mucous membrane
pemphigoid with dapsone. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod. 1999;88:159–163.
-
13. Scully C, Muzio LL. Oral mucosal diseases: mucous
membrane pemphigoid. Br J Oral Maxillofac Surg.
2008;46:358–366.
-
14. Mostafa MI, Hassib NF, Nemat AH. Oral
mucous membrane pemphigoid in a 6-year-old boy:
diagnosis, treatment and 4 years follow-up. Int J
Paediatr Dent. 2010;20:76–79.
-
15. Lozada-Nur F, Miranda C, Maliksi R. Doubleblind clinical trial of 0.05% clobetasol proprionate
ointment in orabase and 0.05% fluocinonide
ointment in orabase in the treatment of patients with
oral vesiculoerosive diseases. Oral Surg Oral Med
Oral Pathol. 1994;77:598–604.
-
16. Vincent SD, Lilly GE, Baker KA. Clinical,
historic, and therapeutic features of cicatricial
pemphigoid: a literature review and open therapeutic
trial with corticosteroids. Oral Surg Oral Med Oral
Pathol. 1993;76:453–459.
-
17. Lamey P-J, Rees T, Binnie W, Rankin K.
Mucous membrane pemphigoid: treatment
experience at two institutions. Oral Surg Oral Med
Oral Pathol. 1992;74:50–53.
-
18. Rogers RS III, Mehregan D, editors. Dapsone
therapy of cicatricial pemphigoid. Semin Dermatol.
1988;7:207–213.
-
19. Helander SD, Rogers RS III. The sensitivity and
specificity of direct immunofluorescence testing in
disorders of mucous membranes. J Am Acad
Dermatol. 1994;30:65–75.
-
20. Scott JE, Ahmed AR. The blistering diseases.
Med Clin North Am. 1998;82:1239-83.
-
21. Suresh L, Kumar V. Significance of IgG4 in the
diagnosis of mucous membrane pemphigoid. Oral
Surg Oral Med Oral Pathol. 2007;104:359-62.
-
22. Neff AG, Turner M, Mutasim DF. Treatment
strategies in mucous membrane pemphigoid. Ther
Clin Risk Manag. 2008;4:617-26.
-
23. Xu HH, Werth VP, Parisi E, Sollecito TP.
Mucous membrane pemphigoid. Dent Clin North
Am. 2013;57(4):611-30.
-
24. Laskaris 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):656-62.
-
25. Bagan J, Jiménez Y, Murillo J, Bagan L. Oral
mucous membrane pemphigoid: a clinical study of
100 low-risk cases. Oral Dis. 2018;24(1-2):132-4.
-
26. Vijayan V, Paul A, Babu K, Madhan B.
Desquamative gingivitis as only presenting sign of
mucous membrane pemphigoid. J Indian Soc
Periodontol. 2016;20(3):340-3.
-
27. Rashid H, Lamberts A, Borradori L, Alberti-Violetti
S, Barry R, 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):1750-64.
-
28. Buajeeb W, Pimolbutr K, Panpradit N, Okuma N.
Oral mucous membrane pemphigoid in a group of Thai
patients – a 15-year retrospective study. J Dent Sci.
2022;17(2):1009-17.
-
29. Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral
mucosal manifestations of autoimmune skin diseases.
Autoimmun Rev. 2015;14(10):930-51.
-
30. Bhogal B, Wojnarowska F, Black M, Xu W, Levene
G. The distribution of immunoglobulins and the C3
component of complement in multiple biopsies from the
uninvolved and perilesional skin in pemphigus. Clin Exp
Dermatol. 1986;11(1):49-53.
-
31. Helander SD, Rogers RS 3rd. The sensitivity and
specificity of direct immunofluorescence testing in
disorders of mucous membranes. J Am Acad
Dermatol. 1994;30(1):65-75.
-
32. Bruch-Gerharz D, Hertl M, Ruzicka T. Mucous
membrane pemphigoid: clinical aspects,
immunopathological features and therapy. Eur J
Dermatol. 2007;17(3):191-200.
-
33. Petruzzi M. Mucous membrane pemphigoid
affecting the oral cavity: short review on
etiopathogenesis, diagnosis and treatment.
Immunopharmacol Immunotoxicol. 2012;34(3):363-
7.
-
34. Neff AG, Turner M, Mutasim DF. Treatment
strategies in mucous membrane pemphigoid. Ther
Clin Risk Manag. 2008;4(3):617-26.
Mukoz Membran Pemfigoidi’nde Ağız Tabanı Tutulumu: Olgu Sunumu
Year 2025,
Volume: 11 Issue: 2, 109 - 114, 29.08.2025
Müberra Deniz
,
Can Aydın
,
Tuğrul Kırtıloğlu
,
Abbas Akbari
Abstract
Amaç:
Mukoz membran pemfigoidi (MMP), mukozal yüzeylerde subepitelyal büllerle seyreden nadir görülen otoimmün bir hastalıktır. Bu olgu sunumunun amacı, mukoz membran pemfigoidinin literatürde nadiren bildirilen ağız tabanı tutulumuna ve bu bölgedeki lezyonların tedavi sürecine dikkat çekmektir.
Gereç ve Yöntemler:
Diş eti kanaması ve periyodik büller şikâyetiyle kliniğimize başvuran 59 yaşındaki kadın hastada, ağız tabanı dahil olmak üzere yaygın oral tutulum mevcuttu. Yapılan detaylı klinik değerlendirme ve histopatolojik inceleme sonucunda MMP tanısı konulmuştur. Hastaya sistemik metilprednizolon, dapson ve mikofenolat mofetil içeren bir tedavi protokolü uygulanmıştır.
Bulgular:
Altı aylık takip sürecinde oral lezyonlarda belirgin gerileme ve klinik iyileşme gözlenmiştir. Tedaviye olumlu yanıt alınmıştır.
Sonuç:
Bu olgu, MMP’nin ağız tabanını da tutabileceğini göstermesi açısından önem taşımaktadır. Erken tanı ve uygun sistemik tedavi ile başarılı sonuçlar elde edilebileceği vurgulanmalıdır.
Ethical Statement
Bu olgu sunumu, Helsinki Bildirgesi ilkeleri doğrultusunda yürütülmüş olup, etik kurallara uygun olarak gerçekleştirilmiştir. Hastadan, olguya ait bilgilerin bilimsel amaçlarla kullanılmasına yönelik onam alınmıştır.
Supporting Institution
Ondokuz Mayıs Üniversitesi, Diş Hekimliği Fakültesi, Periodontoloji Anabilim Dalı
Thanks
Ondokuz Mayıs Üniversitesi Diş Hekimliği Fakültesi Periodontoloji Anabilim Dalı’na verdikleri akademik destek ve katkılarından dolayı teşekkür ederiz.
References
-
1. 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;138:370–
379.
-
2. Ahmed AR, Hombal SM. Cicatricial pemphigoid. Int J
Dermatol. 1986;25:90–96.
-
3. Bagan J, Muzio LL, Scully C. Number III mucous
membrane pemphigoid. Oral Dis. 2005;11:197–218.
-
4. Hofmann SC, Günther C, Böckle BC, Didona D,
Ehrchen J, Gaskins M, et al. S2k guideline for the
diagnosis and treatment of mucous membrane
pemphigoid. J Dtsch Dermatol Ges. 2022;20:1530–1550.
-
5. Du G, Patzelt S, van Beek N, Schmidt E. Mucous
membrane pemphigoid. Autoimmun Rev.
2022;21:103036.
-
6. Srikumaran D, Akpek EK. Mucous membrane
pemphigoid: recent advances. Curr Opin Ophthalmol.
2012;23:523–527.
-
7. Leao J, Ingafou M, Khan A, Scully C, Porter S.
Desquamative gingivitis: retrospective analysis of
disease associations of a large cohort. Oral Dis.
2008;14:556–560.
-
8. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet.
2013;381:320–332.
-
9. Scott JE, Ahmed AR. The blistering diseases. Med
Clin North Am. 1998;82:1239–1283.
-
10. Parker SR, MacKelfresh J. Autoimmune blistering
diseases in the elderly. Clin Dermatol. 2011;29:69–79.
-
11. Broussard KC, Leung TG, Moradi A, Thorne JE,
Fine J-D. Autoimmune bullous diseases with skin and
eye involvement: cicatricial pemphigoid, pemphigus
vulgaris, and pemphigus paraneoplastica. Clin Dermatol.
2016;34:205–213.
-
12. Ciarrocca KN, Greenberg MS. A retrospective study
of the management of oral mucous membrane
pemphigoid with dapsone. Oral Surg Oral Med Oral
Pathol Oral Radiol Endod. 1999;88:159–163.
-
13. Scully C, Muzio LL. Oral mucosal diseases: mucous
membrane pemphigoid. Br J Oral Maxillofac Surg.
2008;46:358–366.
-
14. Mostafa MI, Hassib NF, Nemat AH. Oral
mucous membrane pemphigoid in a 6-year-old boy:
diagnosis, treatment and 4 years follow-up. Int J
Paediatr Dent. 2010;20:76–79.
-
15. Lozada-Nur F, Miranda C, Maliksi R. Doubleblind clinical trial of 0.05% clobetasol proprionate
ointment in orabase and 0.05% fluocinonide
ointment in orabase in the treatment of patients with
oral vesiculoerosive diseases. Oral Surg Oral Med
Oral Pathol. 1994;77:598–604.
-
16. Vincent SD, Lilly GE, Baker KA. Clinical,
historic, and therapeutic features of cicatricial
pemphigoid: a literature review and open therapeutic
trial with corticosteroids. Oral Surg Oral Med Oral
Pathol. 1993;76:453–459.
-
17. Lamey P-J, Rees T, Binnie W, Rankin K.
Mucous membrane pemphigoid: treatment
experience at two institutions. Oral Surg Oral Med
Oral Pathol. 1992;74:50–53.
-
18. Rogers RS III, Mehregan D, editors. Dapsone
therapy of cicatricial pemphigoid. Semin Dermatol.
1988;7:207–213.
-
19. Helander SD, Rogers RS III. The sensitivity and
specificity of direct immunofluorescence testing in
disorders of mucous membranes. J Am Acad
Dermatol. 1994;30:65–75.
-
20. Scott JE, Ahmed AR. The blistering diseases.
Med Clin North Am. 1998;82:1239-83.
-
21. Suresh L, Kumar V. Significance of IgG4 in the
diagnosis of mucous membrane pemphigoid. Oral
Surg Oral Med Oral Pathol. 2007;104:359-62.
-
22. Neff AG, Turner M, Mutasim DF. Treatment
strategies in mucous membrane pemphigoid. Ther
Clin Risk Manag. 2008;4:617-26.
-
23. Xu HH, Werth VP, Parisi E, Sollecito TP.
Mucous membrane pemphigoid. Dent Clin North
Am. 2013;57(4):611-30.
-
24. Laskaris 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):656-62.
-
25. Bagan J, Jiménez Y, Murillo J, Bagan L. Oral
mucous membrane pemphigoid: a clinical study of
100 low-risk cases. Oral Dis. 2018;24(1-2):132-4.
-
26. Vijayan V, Paul A, Babu K, Madhan B.
Desquamative gingivitis as only presenting sign of
mucous membrane pemphigoid. J Indian Soc
Periodontol. 2016;20(3):340-3.
-
27. Rashid H, Lamberts A, Borradori L, Alberti-Violetti
S, Barry R, 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):1750-64.
-
28. Buajeeb W, Pimolbutr K, Panpradit N, Okuma N.
Oral mucous membrane pemphigoid in a group of Thai
patients – a 15-year retrospective study. J Dent Sci.
2022;17(2):1009-17.
-
29. Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral
mucosal manifestations of autoimmune skin diseases.
Autoimmun Rev. 2015;14(10):930-51.
-
30. Bhogal B, Wojnarowska F, Black M, Xu W, Levene
G. The distribution of immunoglobulins and the C3
component of complement in multiple biopsies from the
uninvolved and perilesional skin in pemphigus. Clin Exp
Dermatol. 1986;11(1):49-53.
-
31. Helander SD, Rogers RS 3rd. The sensitivity and
specificity of direct immunofluorescence testing in
disorders of mucous membranes. J Am Acad
Dermatol. 1994;30(1):65-75.
-
32. Bruch-Gerharz D, Hertl M, Ruzicka T. Mucous
membrane pemphigoid: clinical aspects,
immunopathological features and therapy. Eur J
Dermatol. 2007;17(3):191-200.
-
33. Petruzzi M. Mucous membrane pemphigoid
affecting the oral cavity: short review on
etiopathogenesis, diagnosis and treatment.
Immunopharmacol Immunotoxicol. 2012;34(3):363-
7.
-
34. Neff AG, Turner M, Mutasim DF. Treatment
strategies in mucous membrane pemphigoid. Ther
Clin Risk Manag. 2008;4(3):617-26.