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Isolated Laryngeal Involvement in Mucosal Membrane Pemphigoid: Diagnostic and Surgical Treatment Approach

Yıl 2025, Cilt: 35 Sayı: 5, 1041 - 1045, 28.10.2025
https://doi.org/10.54005/geneltip.1771684

Öz

Mucous membrane pemphigoid (MMP) is a chronic autoimmune subepithelial blistering disorder that primarily affects mucosal surfaces. Laryngeal involvement is uncommon but clinically important, as it may result in progressive scarring and severe airway obstruction. Because of its rarity, the diagnosis can be easily overlooked, and the number of reported cases in the literature remains limited. In this case report, we present the clinical findings, diagnostic approach, treatment strategy, and follow-up of a patient with laryngeal MMP. By sharing this case, we aim to contribute to the limited body of evidence on this rare presentation and highlight the importance of early recognition and appropriate management in preventing life-threatening complications.

Kaynakça

  • 1. Hofmann SC, Gunther C, Bockle 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;20:1530-50.
  • 2. Higgins TS, Cohen JC, Sinacori JT. Laryngeal mucous membrane pemphigoid: a systematic review and pooled-data analysis. Laryngoscope 2010;120:529-36.
  • 3. Neff AG, Turner M, Mutasim DF. Treatment strategies in mucous membrane pemphigoid. Ther Clin Risk Manag 2008;4:617-26.
  • 4. Bohelay G, Alexandre M, Le Roux-Villet C, Sitbon I, Doan S, Soued I, et al. Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients. Front Immunol 2022;13:915205.
  • 5. Schmidt E, Rashid H, Marzano AV, Lamberts A, Di Zenzo G, Diercks GFH, et al. European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. J Eur Acad Dermatol Venereol 2021;35:1926-48.
  • 6. Carmel-Neiderman NN, Kaplan I, Oestreicher-Kedem Y. Epiglottitis as the Presenting Sign of Mucous Membranous Pemphigoid: A Case Report. J Voice 2018;32:101-3.
  • 7. Vasiliou A, Nikolopoulos TP, Manolopoulos L, Yiotakis J. Laryngeal pemphigus without skin manifestations and review of the literature. Eur Arch Otorhinolaryngol 2007;264:509-12.
  • 8. Obregon G. Pemphigus of the larynx. Ann Otol Rhinol Laryngol 1957;66:649-55.
  • 9. Stanikova L, Formanek M, Hurnik P, Kantor P, Kominek P, Zelenik K. Diagnosis of Laryngeal Pemphigus Vulgaris Can Be Facilitated Using Advanced Endoscopic Methods. Medicina (Kaunas) 2021;57.
  • 10. Barmatz S, Baniel A, Eremenko R, Neiderman NNC, Wasserzug O, Sprecher E, et al. Laryngeal Pemphigoid Evolution and Response to Treatment. J Voice 2023;37:471 e7- e14.

Mukozal Membran Pemfigoidinde İzole Larenks Tutulumu: Tanısal ve Cerrahi Tedavi Yaklaşımı

Yıl 2025, Cilt: 35 Sayı: 5, 1041 - 1045, 28.10.2025
https://doi.org/10.54005/geneltip.1771684

Öz

Mukoza zarı pemfigoidi (MMP), öncelikli olarak mukozal yüzeyleri etkileyen kronik bir otoimmün subepitelyal büllöz hastalıktır. Larenks tutulumu nadirdir, ancak klinik açıdan önemlidir, çünkü ilerleyici skarlaşma ve ciddi hava yolu tıkanıklığına neden olabilir. Nadir görülmesi nedeniyle tanı kolayca gözden kaçabilir ve literatürde bildirilen vaka sayısı sınırlıdır. Bu olgu sunumunda, larengeal MMP'li bir hastanın klinik bulgularını, tanı yaklaşımını, tedavi stratejisini ve takibini sunuyoruz. Bu vakayı paylaşarak, bu nadir tabloya ilişkin sınırlı sayıdaki kanıta katkıda bulunmayı ve yaşamı tehdit eden komplikasyonları önlemede erken tanı ve uygun tedavinin önemini vurgulamayı amaçlıyoruz.

Kaynakça

  • 1. Hofmann SC, Gunther C, Bockle 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;20:1530-50.
  • 2. Higgins TS, Cohen JC, Sinacori JT. Laryngeal mucous membrane pemphigoid: a systematic review and pooled-data analysis. Laryngoscope 2010;120:529-36.
  • 3. Neff AG, Turner M, Mutasim DF. Treatment strategies in mucous membrane pemphigoid. Ther Clin Risk Manag 2008;4:617-26.
  • 4. Bohelay G, Alexandre M, Le Roux-Villet C, Sitbon I, Doan S, Soued I, et al. Rituximab Therapy for Mucous Membrane Pemphigoid: A Retrospective Monocentric Study With Long-Term Follow-Up in 109 Patients. Front Immunol 2022;13:915205.
  • 5. Schmidt E, Rashid H, Marzano AV, Lamberts A, Di Zenzo G, Diercks GFH, et al. European Guidelines (S3) on diagnosis and management of mucous membrane pemphigoid, initiated by the European Academy of Dermatology and Venereology - Part II. J Eur Acad Dermatol Venereol 2021;35:1926-48.
  • 6. Carmel-Neiderman NN, Kaplan I, Oestreicher-Kedem Y. Epiglottitis as the Presenting Sign of Mucous Membranous Pemphigoid: A Case Report. J Voice 2018;32:101-3.
  • 7. Vasiliou A, Nikolopoulos TP, Manolopoulos L, Yiotakis J. Laryngeal pemphigus without skin manifestations and review of the literature. Eur Arch Otorhinolaryngol 2007;264:509-12.
  • 8. Obregon G. Pemphigus of the larynx. Ann Otol Rhinol Laryngol 1957;66:649-55.
  • 9. Stanikova L, Formanek M, Hurnik P, Kantor P, Kominek P, Zelenik K. Diagnosis of Laryngeal Pemphigus Vulgaris Can Be Facilitated Using Advanced Endoscopic Methods. Medicina (Kaunas) 2021;57.
  • 10. Barmatz S, Baniel A, Eremenko R, Neiderman NNC, Wasserzug O, Sprecher E, et al. Laryngeal Pemphigoid Evolution and Response to Treatment. J Voice 2023;37:471 e7- e14.
Toplam 10 adet kaynakça vardır.

Ayrıntılar

Birincil Dil İngilizce
Konular Kulak Burun Boğaz
Bölüm Case Report
Yazarlar

Bülent Ulusoy 0000-0001-7643-7100

Hürü Beyza Cerit 0009-0006-3113-9947

Gülcan Saylam Kurtipek 0000-0002-3106-4280

Yayımlanma Tarihi 28 Ekim 2025
Gönderilme Tarihi 25 Ağustos 2025
Kabul Tarihi 8 Eylül 2025
Yayımlandığı Sayı Yıl 2025 Cilt: 35 Sayı: 5

Kaynak Göster

Vancouver Ulusoy B, Cerit HB, Saylam Kurtipek G. Isolated Laryngeal Involvement in Mucosal Membrane Pemphigoid: Diagnostic and Surgical Treatment Approach. Genel Tıp Derg. 2025;35(5):1041-5.