Mucous Membrane Pemphigoid

MMP is a chronic autoimmune mucocutaneous disease in which autoantibodies directed at structural proteins of the hemidesmosome destroy the epithelial-connective tissue attachment at the level of the basement membrane, producing a subepithelial separation (24,25). The protein targets of the autoantibodies include BP-1, BP-2, and laminin-5 (epiligrin), all components of the epithelial anchoring apparatus. MMP is a generalized term for a group of closely related disease processes (26). The term "oral mucous membrane pemphigoid" is used if the lesions are confined to the oral mucosa (Fig. 31). The term "cicatricial pemphigoid" is applied to patients with involvement of the ocular mucosa, which produces scarring and may result in blindness. The term "bullous pemphigoid" is

Mucous Membrane Pemphigoid

FIGURE 30 Lichenoid stomatitis in chronic hepatitis C. This red and white lesion at a high-risk site like the lateral border of the tongue was sensitive to spicy food and was highly suspicious for a premalignancy. A biopsy showed a liche-noid mucositis without dysplasia. Note the central erythematous area surrounded by white, radiating striae, the typical "lichenoid" clinical appearance. Source: Courtesy of Stacy Mullins, DDS.

FIGURE 30 Lichenoid stomatitis in chronic hepatitis C. This red and white lesion at a high-risk site like the lateral border of the tongue was sensitive to spicy food and was highly suspicious for a premalignancy. A biopsy showed a liche-noid mucositis without dysplasia. Note the central erythematous area surrounded by white, radiating striae, the typical "lichenoid" clinical appearance. Source: Courtesy of Stacy Mullins, DDS.

FIGURE 31 Mucous membrane pemphigoid. Middle-aged edentulous adult female with history of stomatitis of six months duration involving the upper and lower alveolar ridges. Observe the broad, confluent, ulcerated pseudomembrane-covered zones extending from the crest of the alveolar ridge to the reflection of the labial vestibule. An ophthalmologic workup was negative for ocular mucosal disease involvement. Direct immunofluorescence findings showed IgG and C3 deposits at the basement membrane zone. Source: Courtesy of Charles Dunlap, DDS.

FIGURE 31 Mucous membrane pemphigoid. Middle-aged edentulous adult female with history of stomatitis of six months duration involving the upper and lower alveolar ridges. Observe the broad, confluent, ulcerated pseudomembrane-covered zones extending from the crest of the alveolar ridge to the reflection of the labial vestibule. An ophthalmologic workup was negative for ocular mucosal disease involvement. Direct immunofluorescence findings showed IgG and C3 deposits at the basement membrane zone. Source: Courtesy of Charles Dunlap, DDS.

applied if there is involvement of the skin. Both cicatricial and bullous pemphigoid may be associated with oral mucosal lesions. MMP is commonly seen in middle-aged females.

Differential Diagnosis

Erosive lichen planus, MMP, and pemphigus vulgaris (PV) may have similar clinical features. All may present with desquamative gingivitis (Fig. 32). Frequently in MMP, a gentle stream of air blown tangentially at the surface of the involved gingival mucosa will balloon up the epithelium (Fig. 33). They may be differentiated by DIF testing. In MMP, DIF exhibits a smooth linear deposit of immunoreactants (C3 and IgG) along the basement membrane zone. The Nikolsky sign is positive (Fig. 34). Primary herpes simplex is a febrile illness that exhibits an acute onset of vesicles.

Treatment, Complications, and Prognosis

Depending on the severity of the disease process, topical or systemic corticosteroids may be used. The therapeutic goal should be to provide relief of symptoms while minimizing

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FIGURE 32 Desquamative gingivitis in mucous membrane pemphigoid. Note the gingival erythema and erosion. Desquamative gingivitis is a clinical reaction pattern that is seen in a number of disease processes, including lichen planus, mucous membrane pemphigoid, and pemphigus vulgaris.

FIGURE 33 Desquamative gingivitis in mucous membrane pemphigoid. Note the epithelial separation produced by directing a gentle stream of air at the involved gingiva. This is a sign frequently seen in mucous membrane pemphigoid.

the untoward effects of treatment. Involvement of the ocular mucosa in cicatricial pemphigoid produces scarring and may result in blindness. The lesions may remain localized to the gingiva for a period but generally progress to involve adjacent areas. Treatment of the early lesions may prevent progression of the disease process.

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