|Year : 2013 | Volume
| Issue : 2 | Page : 117-120
Desquamative gingivitis in mucous membrane pemphigoid: A rare case report
Babitha Gungiganur Ajjappa1, Shobha Prakash1, Priya Nagur Karibasappa2
1 Department of Periodontics, College of Dental Sciences, Karnataka, India
2 Department of Oral Pathology and Microbiology, College of Dental Sciences, Karnataka, India
|Date of Web Publication||4-Jul-2014|
Priya Nagur Karibasappa
Reader Room No. 7, Department of Oral and Maxillofacial Pathology and Microbiology, College of Dental Sciences, Davangere - 577 004, Karnataka
Source of Support: None, Conflict of Interest: None
Mucous membrane pemphigoid (MMP) is a relatively rare chronic vesiculobullous disease that affects the mucosal surfaces, including the conjunctiva, oral cavity, larynx, esophagus, upper respiratory tract and genitalia as well as the skin. Recurrent lesions result in permanent scarring that causes morbidity and mortality. Desquamative gingivitis is the most characteristic feature of MMP and may mark the onset of the disease. In this article, we report the clinical manifestations, histological features and treatment of a case of MMP.
Keywords: Biopsy, desquamative gingivitis, mucous membrane pemphigoid, treatment
|How to cite this article:|
Ajjappa BG, Prakash S, Karibasappa PN. Desquamative gingivitis in mucous membrane pemphigoid: A rare case report. Int J Oral Health Sci 2013;3:117-20
|How to cite this URL:|
Ajjappa BG, Prakash S, Karibasappa PN. Desquamative gingivitis in mucous membrane pemphigoid: A rare case report. Int J Oral Health Sci [serial online] 2013 [cited 2019 Sep 19];3:117-20. Available from: http://www.ijohsjournal.org/text.asp?2013/3/2/117/135990
| Introduction|| |
Mucous membrane pemphigoid (MMP) or cicatricial pemphigoid (CP) is an autoimmune blistering disease that predominantly affects the mucous membranes, including the mouth, oropharynx, the conjunctiva, the nares and the genitalia. It is associated with auto-antibodies directed against basement membrane zone target antigens. Auto-antibodies of the IgG subclass, particularly IgG 4 , are associated with CP. However, IgA antibodies have also been detected. The two major antigens associated with CP are bullous pemphigoid antigen 2 (BPAG2) and epiligrin (laminin-5). The primary lesions of MMP occur when auto-antibodies directed against proteins in the basement membrane zone, acting with complement (C3) and neutrophils, cause a sub-epithelial split and subsequent vesicle formation. The antigens associated with MMP are most frequently present in the lamina lucida portion of the basement membrane, but recent research has demonstrated that the identical antigen is not involved in all cases and the lamina densa may be the primary site of involvement in some cases. The circulating auto-antibodies are not the same in all cases, and subsets of MMP have been identified by the technique of immunofluorescent staining of the skin that has been split at the basement membrane zone with the use of sodium chloride.  The majority of cases of MMP demonstrate IgG directed against antigens on the epidermal side of the salt-split skin, which have been identified as BP 180 (also called type XVII collagen); however, cases of MMP have also been identified where the antigen is present on the dermal side of the split. This latter antigen has been identified as epiligrin (laminin 5), an adhesion molecule that is a component of the anchoring filaments of the basement membrane. ,
MMP chiefly affects the oral cavity, larynx, esophagus and ocular membranes, but rarely the skin.  Ocular lesions have been reported to occur in 11-61% of patients with MMP, while skin lesions occur in 0-11% of patients. , The oral cavity usually represents the first and often the only site of disease involvement. Intraoral manifestations of MMP include desquamative gingivitis, vesiculobullous lesions and ulcerations. Orally, it primarily affects the gingival, but may involve any area. The disease is seen twice as often in women, primarily those middle aged and older.  The identification of the underlying pathosis of the gingival lesions is of utmost importance.
This report describes a case of MMP in which the lesions were confined to the gingiva. Clinical signs, symptoms and histological characteristics are discussed herein.
| Case Report|| |
A 61-year-old male patient was referred to the Department of Periodontology, College of Dental Sciences, Davangere, Karnataka, India by his physician. He complained of burning sensation and soreness of the gums since 3 months and aggravation of pain on having spicy foods. Systemically, the patient was healthy without any adverse habits like smoking.
On intraoral examination, localized desquamation along with diffuse white patchy gray areas on the marginal, attached and interdental gingiva in the anterior and posterior areas of the maxillary palatal gingiva and posterior areas buccally in relation to maxillary and mandibular arches was present. The gingival surface was smooth with slight pitting in areas on pressure. The gingival epithelium could easily be peeled from the underlying connective tissue (Nikolsky's sign positive) [Figure 1] and [Figure 2].
|Figure 2: Facial view exhibiting areas of desquamation on posterior region of mandibular buccal gingiva|
Click here to view
An extra-oral examination of the patient revealed numerous skin lesions on his body, especially face, hands and neck, since 1 year. The skin lesions started as small water-filled boils, which healed after few days leaving behind a scar. The occurrence of the skin lesions aggravated on exposure to sun [Figure 3].
A biopsy of lesional tissue was obtained from the palatal gingiva adjacent to the left maxillary first premolar region. The biopsied tissue was submitted for microscopic examination in a container containing 10% formalin. Light microscopic examination of the section stained with hematoxylin and eosin revealed a hyperparakeratinized stratified squamous epithelium overlying the connective tissue. The rete ridges were broad and blunt in areas with discontinuous subepithelial split. The underlying connective tissue showed loose connective tissue stroma with mild, mixed inflammatory infiltrate predominantly with neutrophils, lymphocytes and few eosinophils [Figure 4]. The patient had no ocular changes. On the basis of clinical and histopathological features, the diagnosis of MMP was made. The patient had widespread involvement of the gingival, which may be worsened with poor oral hygiene. Therefore, the periodontal management was also considered as an essential part in the overall treatment. The patient was motivated for plaque control, and the initial phases (scaling and root planing) of periodontal treatment were performed to eliminate the local irritants. The patient was instructed to use a soft bristled toothbrush and 3% hydrogen peroxide (1 H 2 O 2 :2 H 2 O) mouthwash two times a day and topical steroid (0.1% triamcinolone) was prescribed for application over the oral lesions three times a day for 3 weeks. The lesions improved considerably. The patient was prescribed systemic corticosteroid therapy by the dermatologist (Prednisone 30-40 mg daily and gradually reduced to a daily maintenance dose of 5-10 mg).
The patient was reviewed every 2 weeks for the first month; the lesions had subsided within 4 weeks. The patient was instructed regarding oral hygiene maintenance. Because these lesions can recur, the patient is under regular observation.
| Discussion|| |
MMP is a group of uncommon chronic blistering diseases found in 2-5 people per 100,000 population a year.  In the past, MMP was known by several terms, including benign mucous membrane pemphigoid, CP and ocular or oral gingival pemphigoid. However, in reporting the results of the first international consensus on MMP, Chan and others  recommended the term "mucous membrane pemphigoid" because the disease may not be benign when it causes blindness from ocular involvement or death from laryngeal scarring, it may not be scarring, as in gingival involvement, and it may affect a number of mucous membranes such as the oral and nasal mucosa, pharynx, anus, genital mucosa, esophagus and trachea. The skin may be affected, but is always a minor component.
The disease occurs nearly twice as frequently in females as it does in males, with the peak age and involvement being between 40 and 50 years. Typically, the vesiculobullous lesions occur on the oral mucous membranes and conjunctiva. Lesions also occur on the skin, particularly around the genitalia and near the body orifices in about 25% of cases. Typically, these lesions heal by scar formation, particularly on the conjunctiva. Other mucous membrane surfaces may be involved such as the nose, larynx, pharynx, esophagus, vulva, vagina, penis and anus.
The clinical presentation in this patient intraorally was desquamative gingivitis with the areas of erosive desquamation, ulceration and vesiculation of the attached gingival, which are the classic features of desquamative gingivitis. The lesions were confined to the gingiva and skin and there were no accompanying ocular lesions. Lesions on the skin featured with bullae that had ruptured leaving a scar. Light microscopic examination of the biopsy specimen revealed areas with sub-epithelial split and loose fibrous connective tissue with a mild chronic inflammatory cell infiltrate. These clinical and histopathological features present in this male patient aged 61 years was highly suggestive of MMP. Other vesiculobullous diseases such as bullous pemphigoid, pemphigus, erythema multiforme and bullous lichen planus were considered for differential diagnosis.
The bullous pemphigoid primarily affects the skin while CP characteristically involves the eyes as well as the oral mucosa and/or skin.  In contrast to MMP, the basement membrane remains attached to the connective tissue rather than to the overlying separated epithelium. 
Pemphigus is similar in its clinical presentation to MMP, but it produces acantholysis with cleavage of the spinous cell layer. In pemphigus, the vesicle is intraepithelial whereas in MMP, due to the separation of connective tissue from the epithelium, the vesicle is located sub-epithelially. ,
In lichen planus, the inflammatory infiltrate is non-specific in nature consisting of lymphocytes, plasma cells and neutrophils.  Microscopic evaluation of the oral lesions reveals hydropic degeneration of the basal layer of the oral epithelium.  Erythema multiforme is an inflammatory disease involving the skin and oral mucosa. Although extensive bullous and ulcerative lesions may develop in this condition, desquamative gingivitis is not seen. Microscopic analysis of an oral lesion also reveals an unusual degeneration of the upper stratum spinosum of the oral epithelium, which is specific for the entity. ,
Management of MMP depends on the severity of the signs and symptoms of the disease. Systemic or topical corticosteroid is usually prescribed to the patients with MMP along with motivation for plaque control and oral hygiene instruction.
The diagnosis of MMP depends on taking a careful clinical history and performing a thorough clinical examination. However, the definitive diagnosis depends on the histopathologic examination of gingival tissue. However, immunofluorescent examination for identifying the underlying cause of desquamative gingivitis is required.
| Acknowledgement|| |
I wish to acknowledge my heartful thanks to, Dr. Kunal Dhingra, for the tremendous help in preparing this manuscript without whose help this manuscript would not have been possible.
| References|| |
|1.||Chan LS, Hammerberg C, Cooper KD. Cicatricial pemphigoid. Identification of two distinct sets of epidermal antigens by IgA and IgG class circulating autoantibodies. Arch Dermatol 1990;126:1466-8. |
|2.||Allbritton JI, Nousari HC, Anhalt GJ. Anti-epiligrin (laminin 5) cicatricial pemphigoid. Br J Dermatol 1997;137:992-6. |
|3.||Nousari HC, Rencic A, Hsu R, Yancey KB, Anhalt GJ. Anti-epiligrin cicatricial pemphigoid with antibodies against the gamma2 subunit of laminin 5. Arch Dermatol 1999;135:173-6. |
|4.||Weinberg MA, Insler MS, Campen RB. Mucocutaneous features of autoimmune blistering diseases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1997;84:517-34. |
|5.||Lamey PJ, Rees TD, Binnie WH, Rankin KV. Mucous membrane pemphigoid. Treatment experience at two institutions. Oral Surg Oral Med Oral Pathol 1992;74:50-3. |
|6.||Shklar G, McCarthy PL. Oral lesions of mucous membrane pemphigoid. A study of 85 cases. Arch Otolaryngol 1971;93:354-64. |
|7.||Dayan S, Simmons RK, Ahmed AR. Contemporary issues in the diagnosis of oral pemphigoid: A selective review of the literature. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88:424-30. |
|8.||Challacombe SJ, Setterfield J, Shirlaw P, Harman K, Scully C, Black MM. Immunodiagnosis of pemphigus and mucous membrane pemphigoid. Acta Odontol Scand 2001;59:226-34. |
|9.||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;138:370-9. |
|10.||Position paper: Oral features of mucocutaneous disorders. J Periodontol 2003;74:1545-56. |
|11.||Shafer WG, Hine MK, Levy BM. Diseases of periodontium and Diseases of skin. In: A Textbook of Oral Pathology, 4 th ed. Philadelphia: WB Saunders Publisher; 1983. p. 795-6, 835-6. |
|12.||Manton SL, Scully C. Mucous membrane pemphigoid: An elusive diagnosis? Oral Surg Oral Med Oral Pathol 1988;66:37-40. |
|13.||Rogers RS 3 rd , Seehafer JR, Perry HO. Treatment of cicatricial (benign mucous membrane) pemphigoid with dapsone. J Am Acad Dermatol 1982;6:215-23. |
|14.||Vincent SD, Fotos PG, Baker KA, Williams TP. Oral lichen planus: The clinical, historical, and therapeutic features of 100 cases. Oral Surg Oral Med Oral Pathol 1990;70:165-71. |
|15.||Leigh IM, Mowbray JF, Levene GM, Sutherland S. Recurrent and continuous erythema multiforme - a clinical and immunological study. Clin Exp Dermatol 1985;10:58-67. |
|16.||Shklar G. Desquamative gingivitis and oral mucous membrane disease. In: Carranza FA, Newman GM, editors. Clinical Periodontology, 8 th ed. Philadelphia: Saunders Publisher; 1996. p. 259-75. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4]