Thomas Nirupa1, Venugopal Lekshmi1, Steaphen Paul2, George Eldhose2

 1Department of Oral Pathology, Annoor Dental College and Hospital, Muvattupuzha

2Department of Oral & Maxillofacial Surgery, Annoor Dental College and Hospital, Muvattupuzha.

Running title – Mucous membrane pemphigoid

Received: 11-01-2024

Revised: 15-01-2024

Accepted: 22-01-2024

Address for correspondence: Dr. Nirupa Thomas, Kaithapillil (H), Ramamangalam P.O, Ramamangalam, Kerala, India

E-mail-nirupathomas@gmail.com

This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-Noncommercial ShareAlike 4.0 license, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms

How to cite this article: Thomas N, Venugopal L, Steaphen P, George E. A case report of Mucous membrane pemphigoid in a 17 year old female. J Oral Biomed Sci 2024; 3(1):13-16

Abstract

Mucous membrane Pemphigoid is a heterogenous group of autoimmune  subepithelial blistering disorder involving mucous membrane. The disease has got a strong female predominance with the peak of age involvement being the 5th decade. Typically the mucosal lesions occur on the oral mucous membrane and conjunctiva. Here we present a case of mucous membrane pemphigoid occurring in a female patient during her adolescent period.

Key words: Mucous membrane pemphigoid, autoimmune, subepithelial blistering

INTRODUCTION

Autoimmune blistering disorders are a class of rare skin disorders that happen when the immune system attacks skin and mucous membrane. It includes a number of subepithelial blistering disorders such as pemphigus, pemphigoid, IgA  mediated bullous dermatoses and epidermolysis bullosa acquisita.1 Mucous membrane pemphigoid is an autoimmune blistering disease that predominantly affects mucous membrane, including the mouth and oropharynx, conjunctiva ,nares and the genitalia.. Skin involvement is restricted to the regions of head, neck, and upper torso. Patients with cutaneous involvement present with tense blisters and erosions where the  blisters heal with scarring and pigmentation. Sequelae of mucosal involvement include decreased vision, blindness and supraglottic stenosis with hoarseness or airway obstruction.2 Elderly females are commonly affected with mean age onset of 50–80 years. Intraoral features of MMP include desquamative gingivitis, vesicles, erosions covered by pseudomembranes and ulcers.3 MMP related autoantigens include BP180, BP230, laminin 332, type VII collagen among which autoantibodies BP180  and LM 332 have been most often reported.4

CASE REPORT

A 17-year-old female patient presented with 2 year history of blister formations and  ulcerations in the oral cavity. The patient experienced pain and a burning sensation when consuming hot and spicy foods. Although a physician prescribed a topical antiseptic gel for the ulcers, it provided no relief upon application. There was no relevant personal history, and no systemic disease was reported.

An extraoral examination revealed no lesions in the eyes, skin, or genital areas. Intraoral examination showed generalized gingival erythema on both the labial and palatal surfaces of the maxillary and mandibular gingiva. Additionally, the maxillary gingiva exhibited a loss of stippling from the attached gingiva to the mucogingival junction on both the labial and palatal aspects.Single mucosal erosions seen in labial mucosa in relation to 24,25 region. [fig:1,fig: 2].The entire oral mucosa was tender on palpation. The provisional diagnosis given was Bullous pemphigus.

Figure 1& 2 showing mucosal erosions in the palatal and labial mucosa

An incisional biopsy was performed on the labial mucosa in relation to 23 region and palatal mucosa in relation to 24,25 regions and sent for histopathological examination. The laboratory received 2 bits of formalin fixed tissue of size 8×5 mm and 5x6mm, which were firm in consistency.

Microscopically, keratinized stratified squamous epithelium of variable thickness with subepithelial split was observed. The underlying connective tissue stroma showed moderately dense inflammatory cell infiltrate predominantly lymphocytes and capillary vessels .[fig:3,fig:4] The clinical and histopathological features favor a confirmatory diagnosis of MMP.

Figure 3 & 4 showing keratinized epithelium of variable thickness with subepithelial split

DISCUSSION

Mucous membrane pemphigoid (MMP) is a chronic, autoimmune vesiculobullous disease mainly affects   mucosal tissues and, rarely seen in the skin. Mucous membrane pemphigoid was first reported by Wichmanns and Thost 5. In the past, MMP was referred to as “benign mucous membrane pemphigoid,” “cicatricial pemphigoid” and “ocular or oral–gingival pemphigoid.”6 In the literature on MMP, involvement of the skin is rare, but the conjunctiva may sometimes be affected, depending on the severity of the disease, and women are affected more than men. In our case also there were no skin lesions accompanying oral lesions. It is usually seen in adults over 40 years of age and occasionally seen in children. In our case the patient was a young female aged 17 years. There was no predisposing factors such as emotional stress, smoking, nutritional deficiency or systemic disease in that patient.7

The aetiology of MMP remains unclear. Severe mucosal inflammatory injury, medications (clonidine, D-penicillamine, indomethacin) viruses, UV light, and occasional occurrence with other autoimmune diseases, and genetic predispositions such as HLADQB1 are some of the suspected aetiological factors. . 8,9

The most frequently presenting feature in MMP is desquamation of gingiva but other sites such as conjunctiva, nasal, esophageal, laryngeal, vaginal mucosa as well as skin may be involved.10 There is no evidence in cited literature supporting the association of MMP and post-menopausal condition or habit of smoking.11

Many diseases, including bullous pemphigoid, pemphigus vulgaris and bullous lichen planus, should be considered in the differential diagnosis of MMP. The lesions of bullous pemphigoid and MMP closely resemble each other, the main difference being in their clinical presentation. The clinical course in patients with bullous pemphigoid is limited but the course in patients with MMP is usually protracted and progressive.10

The vesicular lesions of MMP are also similar to those of pemphigus vulgaris, particularly when the lesion is confined to the oral cavity. The lesions of

pemphigus vulgaris  appears  first in the mouth, but soon spread widely on the skin. Acantholysis and intra-epithelial clefting are seen, and immunofluorescence reveals immunoglobulin G auto-antibody bound around the surface of the prickle cells in the epithelium, but in MMP, the G –auto antibody is bound along the basement membrane.12

On clinical examination bullous-type oral lichen planus may be confused with MMP. However, the histopathological subepithelial separation without degeneration of the basal layer of the epithelium seen in MMP is indicative in making the differential diagnosis. In bullous-type oral lichen planus, juxta epithelial inflammatory cell infiltration can be seen histopathologically. In addition, the presence of the characteristic clinical white striations at the periphery of the ulcerated areas in lichen planus helps to differentiate between the two conditions.13

Once the diagnosis has been established by microscopy and direct immunoflorescence ,we can use topical corticosteroids, either alone or in association with systemic corticosteroids for treatment.14 If topical corticosteroids are unsuccessful , Immunosuppressive drugs such as methotrexate, azathioprine, levamisole, cyclophosphamide, and mycophenolate  mofetil are  used along with systemic corticosteroids.15 Promising and dramatic responses have been achieved with Dapsone. Patient should be subjected to regular blood profile testing as prolonged dapsone use may induce hemolytic anemia.16Patients with  less severe disease  has also been treated with a tetracycline derivative or a combination of tetracycline and niacinamide.17 Before choosing which medication to use, the oral hygiene of the patient should be improved. Intravenous immunoglobulins, plasmapheresis, and Low level laser therapy (LLLT) constitute the recent development in the management of MMP.18

Conflict of interest: None

Source of support: Nil

REFERENCES

  1. Scully C, Carrozzo M, Gandolfo S, Puiatti P, Monteil R. Update on mucous membrane pemphigoid: A heterogeneous immune-mediated subepithelial blistering entity. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 1999;88:56–68.
  2. Xu HH, Werth VP, Parisi E, Sollecito TP. Mucous membrane pemphigoid. Dent Clin North Am. 2013;57:611–30
  3. Schmidt E, Zillikens D. Pemphigoid diseases. Lancet. 2013;381:320–32
  4. Bruch-Gerharz D, Hertl M, Ruzicka T. Mucous membrane pemphigoid: Clinical aspects, immunopathological features and therapy. Eur J Dermatol. 2007;17:191–200.
  5. Wichmanns JE: Ideen zur Diagnostik. Hanover, Helwig. 1: p 89 (1894)
  6. Ciarrocca KN, Greenberg MS. A retrospective study of the management of mucous membrane pemphigoid with dapsone. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999; 88(2):159–63.
  7. Jolliffe DS, Sim-Davis D: Cicatricial pemphigoid in a young girl: report of a case. Clin Exp Dermatol 1977; 2: 281 – 284.
  8. Nayar M, Wojnarowska F, Vemning V, Taylor CJ. Association of autoimmunity and cicatricial pemphigoid: is there an immunogenetic basis?? J Am Acad Dermatol; 25: 1011-1015 (1991).
  9. Yunis JJ, Mobini N, Yunis EJ, Alper CA, Deulofeut R, Rodriguez A, Foster CS, Marcus-Bagley D, Good RA, Ahmed AR.Major histocompatibility complex class II markers in clinical variants of cicatricial pemphigoid. Proc
  10. Neville, B.W., Damm, D.D., Allen et al . Oral & Maxillofacial Pathology. 4th Edition(2016)
  11. Delgado JC, Tarbay D, Yunis EJ et al. A common major histocompatibility complex class II allele HLA-DQB1*0301 is present in clinical variant of pemphigoid. Proc Natl Acad Sci USA.; 93: 8569-8571 (1996).
  12. Cawson RA, Odell EW: Principals of investigation and diagnosis. In: Essentials of Oral Pathology and Oral Medicine, 6th edn. London: Churchill Livingstone, 1998; pp1 – 15.
  13. Shklar G: Desquamative gingivitis and oral mucous membrane diseases. In: Clinical Periodontology, 8th edn (Carranza FA, Newman GM, eds). Philadelphia: WB Saunders, 1996; pp259 – 275.
  14. Lamey PJ, Rees TD, Binnie WH, Rankin KV. Mucousmembrane pemphigoid–Treatment experience at twoinstitutions. Oral Surg Oral Med Oral Pathol; 74:50-53 (1992).
  15. Lever WF. Pemphigus and pemphigoid. A review ofthe advances made since 1964. Am J Dermatopathol; 1: 2-32 (1979).
  16. Rogers RS III, Seehafer JR, Perry HO. Treatment ofcicatricial (benign mucous membrane) pemphigoidwith dapsone. J Am Acad Dermatol; 6: 215-223 (1982).
  17. Thornfeldt CR, Menkes AW. Bullous pemphigoid controlledby tetracycline. J Am Acad Dermatol; 16:305-310 (1987).
  18. Damoulis PD, Gagari E. Combined treatment ofperiodontal disease and benign mucous membranepemphigoid. Case report with 8 years maintenance.J Periodontol; 71: 1620-1629 (2000).

Journal