A retrospective analysis of oral autoimmune bullous diseases at a Thai oral medicine center

Abstract

Background: /Purpose Autoimmune bullous diseases (AIBDs) are rare conditions that can affect daily life and be life-threatening. However, there is scant research on Thai patients with oral AIBDs.

Materials and methods: Retrospective analysis of the characteristics, distribution, and treatment outcomes of oral AIBDs in Thai patients (20-year period).

Results: Eighty-two oral AIBDs patients were diagnosed, mostly female ages ranging from middle-aged to elderly. The most common subtype was pemphigus vulgaris (PV) (59.8 %), followed by mucous membrane pemphigoid (MMP) (26.8 %), bullous pemphigoid (BP) (8.5 %), and linear IgA bullous dermatosis (LABD) (4.9 %). The gingiva was the most affected site. Notably, 81.8 % of MMP patients had only oral lesions (mostly gingiva), while most PV patients had multiple lesions, with oral lesions often appearing first. Corticosteroids (CS) (topical and/or systemic) were the primary treatment. The overall response rate for control of disease activity (CDA) was 73.9 %, with complete remission (CR) in 17.4 %. Patients receiving only topical CS had fewer side effects (66.7 % reported none, others mild) compared with combined therapy (48.6 % reported side effects).

Conclusion: PV was the most prevalent subtype, often presenting with oral and skin lesions, with oral lesions appearing first. Conversely, MMP primarily manifested as isolated oral lesions, often with desquamative gingivitis. Combination therapy with topical and systemic CS was the most common treatment for oral AIBDs. Notably, patients treated with topical CS only experienced significantly fewer and milder side effects compared with those receiving other treatments.

Keywords: Linear IgA bullous dermatosis; Mucous membrane pemphigoid; Oral autoimmune bullous diseases; Pemphigoid; Pemphigus vulgaris; Thai.

Figure 1

Conceptual framework. Abbreviations: DIF, direct immunofluorescence microscopy; ELISA, enzyme-linked immunosorbent assay test; IIF, indirect immunofluorescence microscopy; Oral AIBDs, Oral autoimmune bullous diseases.

Figure 2

Clinical presentation and microscopy examination of a patient with PV. (A, B, C and D) A 74-year-old male with PV presented with extensive erosions and ulcers on the oral mucosa. (E) The same patient, who has lesions exhibiting crusted erosions on the shoulder. Image courtesy of Associate Professor Pornpan Piboonratanakit, Oral Medicine Department, FDCU. (F, G and H) Microscopy examination of a 56-year-old female with PV. (F) Histopathological examination shows suprabasal split formation. (G and H) DIF microscopy shows intercellular deposition of C3 (G) and IgG (H) (Chicken wire pattern) in the epithelium of the oral mucosa. Image courtesy of Dr.Keeratika Wongtim, Oral Medicine Department, FDCU. Abbreviations: C3, complement 3; DIF, direct immunofluorescence; FDCU, Faculty of Dentistry, Chulalongkorn University; IgG, immunoglobulin G; PV, pemphigus vulgaris.

Figure 3

Clinical presentation and microscopy examination of a 57-year-old male with oral MMP. (A) At the first visit, localized ulcerations were observed on the lingual gingiva of the lower teeth. (B) Complete remission of lesions after treatment with only topical CS (Initial treatment with triamcinolone acetonide 0.1 % mouthwash, followed by a combination of fluocinolone acetonide 0.1 %/clotrimazole 1 % gel). The oral status after 12-month follow-up. (C) Histopathological examination shows sub-basilar split formation. (D and E) DIF microscopy shows linear IgG (D) and C3 (E) along the BMZ. Image courtesy of Emeritus Professor Kobkan Thongprasom, Oral Medicine Department, FDCU. Abbreviations: BMZ, basement membrane zone; C3, complement 3; CS, corticosteroids; DIF, direct immunofluorescence; FDCU, Faculty of Dentistry, Chulalongkorn University; IgG, immunoglobulin G; MMP, mucous membrane pemphigoid.

Figure 4

Clinical presentation and DIF examination of a 22-year-old female with oral BP. (A) At the first visit, erosions and ulcers were observed on the buccal gingiva of upper and lower posterior teeth. (B) Complete remission of lesions after treatment with topical and systemic CS. The oral status after 12-month follow-up. (C) DIF microscopy demonstrates a linear deposition of C3 along the BMZ. Image courtesy of Emeritus Professor Kobkan Thongprasom, Oral Medicine Department, FDCU. Abbreviations: BMZ, basement membrane zone; BP, bullous pemphigoid; C3, complement 3; CS, corticosteroids; DIF, direct immunofluorescence; FDCU, Faculty of Dentistry, Chulalongkorn University.

Figure 5

Clinical presentation and microscopy examination of a 46-year-old male with oral LABD. (A) At the first visit, desquamative gingivitis and ruptured bullae were observed. (B) Complete remission of lesions after treatment with only topical CS (fluocinolone acetonide 0.1 % mouthwash). The oral status after 6-month follow-up. (C) Histopathological examination indicates sub-basilar separation. (D) DIF microscopy demonstrates linear IgA deposition along the BMZ. Image courtesy of Assistant Professor Titipong Prueksrisakul, Oral Medicine Department, FDCU. Abbreviations: BMZ, basement membrane zone; CS, corticosteroids; DIF, direct immunofluorescence; FDCU, Faculty of Dentistry, Chulalongkorn University; IgA, immunoglobulin A; LABD, linear IgA bullous dermatosis.