Mucous membrane pemphigoid – a report of four cases

  • Ivana Jocić Military Medical Academy, Department of Dermatology and Venereology
  • Dragana Daković Military Medical Academy, Department of Dentistry
  • Lidija Kandolf Sekulović Military Medical Academy, Department of Dermatology and Venereology
  • Ljubomir Novaković Queen Elizabeth Hospital, Lewisham and Greenwich NHS Trust & St John's Institute of Dermatology, Guy's and St Thomas' NHS Trust, London, United Kingdom
  • Željko Mijušković Military Medical Academy, Department of Dermatology and Venereology
Ključne reči: dijagnoza;, pemfigoid, bulozni;, elisa;, fluorescentna antitela, direktna tehnika;, remisija, indukcija

Sažetak


Introduction. Mucous membrane pemphigoid (MMP) is a rare autoimmune, chronic inflammatory disease that affects mucous membranes, most commonly the eyes and mouth, with or without skin involvement. It is a complex disease with several complications, including scarring, especially on conjunctival mucosa, that can lead to visual loss. Case report. We report four patients (two men and two women) with MMP. In all patients, the disease started between seventy and eighty years of age. The diagnosis was confirmed based on clinical appearance, histology, direct and indirect immunofluorescence studies, indirect split skin technique, and enzyme-linked immunosorbent assay (ELISA) test. The majority of lesions were on the gums and buccal mucosa; one patient had laryngeal involvement and a lesion on the umbilicus. No ocular involvement and no malignancy were detected. Direct immunofluorescence tests revealed continuous linear IgG deposition in the basal membrane zone in two patients, and they were treated with oral nicotinamide and tetracycline hydrochloride. In two patients, we detected IgG along with IgA linear deposition; they received treatment with methylprednisolone. Complete remission was achieved in all patients. Conclusion. Early diagnosis and an adequate therapeutic approach are necessary for the MMP treatment in long-term disease control and reduction of disease-related complications.

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1.      Madgar O, Baniel A, Yarom N, Glikson E, Zeeli T, Sprecher E, et al. Mucous membrane pemphigoid–otorhinolaryngological manifestations: a retrospective cohort study. Eur Arch Otorhinolaryngol, 2020; 277(3): 939–45.

2.      Ormond M, McParland H, Thakrar P, Donaldson ANA, Andiappan M, Cook RJ, et al. Validation of an Oral Disease Severity Score (ODSS) tool for use in oral mucous membrane pemphigoid. Br J Dermatol 2020; 183(1): 78‒85.

3.      Kamaguchi M, Iwata H. The Diagnosis and Blistering Mechanisms of Mucous Membrane Pemphigoid. Front Immunol 2019; 10: 34.

4.      Lee J, Seiffert-Sinha K, Attwood K, Sinha AA. A Retrospective Study of Patient-Reported Data of Bullous Pemphigoid and Mucous Membrane Pemphigoid From a US-Based Registry. Front Immunol 2019; 10: 2219.

5.      La Placa M, Balestri R, Tartari F, Sechi A, Ferrara F, Loi C, et al. Mucous Membrane Pemphigoid-Associated Malignancies: Case Series and a Brief Overview of the Literature. Dermatol Pract Concept 2019; 9(2): 119‒25.

6.      Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev 2015; 14(10): 930‒51.

7.      Shruthi R, Shruthi H, Vidya A, Subhas B, Sajad AB. Pemphigus vulgaris presenting as bleeding gums: A case report. J Turgut Ozal Med Cent 2017; 24(3): 324‒7.

8.      Georgoudis P, Sabatino F, Szentmary N, Palioura S, Fodor E, Hamada S, et al. Ocular Mucous Membrane Pemphigoid: Current State of Pathophysiology, Diagnostics and Treatment. Ophthalmol Ther 2019; 8(1): 5‒17.

9.      Wickmanns JE. Ideas on the diagnosis. Hanover: Helwig; 1894. p. 89.

10.   Arduino PG, Broccoletti R, Carbone M, Conrotto D, Pettigiani E, Giacometti S, et al. Describing the gingival involvement in a sample of 182 Italian predominantly oral mucous membrane pemphigoid patients: A retrospective series. Med Oral Patol Oral Cir Bucal 2017; 22(2): e149–52.

11.   Di Zenzo G, Carrozzo M, Chan LS. Urban legend series: mucous membrane pemphigoid. Oral Dis 2014; 20(1): 35‒54.

12.   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(3): 370‒9.

13.   Neff AG, Turner M, Mutasim DF. Treatment strategies in mucosus membrane pemphigoid. Ther Clin Risk Manag 2008; 4(3): 617–26.

14.   Hasan S. Desquamative gingivitis - A clinical sign in mucous membrane pemphigoid: Report of a case and review of literature. J Pharm Bioallied Sci 2014; 6(2): 122‒6.

15.   Santi CG, Gripp AC, Roselino AM, Mello DS, Gordilho JO, Marsillac PF, et al. Consensus on the treatment of autoimmune bullous dermatoses: bullous pemphigoid, mucous membrane pemphigoid and epidermolysis bullosa acquisita - Brazilian Society of Dermatology. An Bras Dermatol 2019; 94(2 Suppl 1): 33‒47.

16.   Egan CA, Lazarova Z, Darling TN, Yee C, Yancey KB. Anti-epiligrin cicatricial pemphigoid: clinical findings, immunopathogenesis, and significant associations. Medicine (Baltimore) 2003; 82(3): 177‒86.

17.   Ujiie H, Iwata H, Yamagami J, Nakama T, Aoyama Y, Ikeda S, et al. Committee for Guidelines for the Management of Pemphigoid Diseases (Including Epidermolysis Bullosa Acquisita). Japanese guidelines for the management of pemphigoid (including epidermolysis bullosa acquisita). J Dermatol 2019; 46(12): 1102‒35.

18.   Witte M, Zillikens D, Schmidt E. Diagnosis of Autoimmune Blistering Diseases. Front Med (Lausanne) 2018; 5: 296.

19.   Feliciani C, Joly P, Jonkman MF, Zambruno G, Zillikens D, Ioannides D, et al. Management of bullous pemphigoid: the European Dermatology Forum consensus in collaboration with the European Academy of Dermatology and Venereology. Br J Dermatol 2015; 172(4): 867‒77.

20.   Sacher C, Hunzelmann N. Cicatricial pemphigoid (mucous membrane pemphigoid): current and emerging therapeutic approaches. Am J Clin Dermatol 2005

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2023/02/13
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