Benign Mucosal Pemphigoid

Definition

Definition of Benign Mucosal Pemphigoid
Cicatricial pemphigoid (also known as "Benign mucosal pemphigoid," "Benign mucous membrane pemphigoid," "Ocular pemphigus," and "Scarring pemphigoid") is a rare chronic autoimmune subepithelial blistering disease characterized by erosive skin lesions of the mucous membranes and skin that results in scarring of at least some sites of involvement.

Symptoms

Symptoms of Benign Mucosal Pemphigoid
Diagnostic techniques:

1. Antibodies (IgG) precipitates complement (C3) in the lamina lucida of the basement membrane.
2. Circulating auto-antibodies to BP-1 antigen (located in hemidesmosome). 50% have BP-2.
3. Positive Nikolsky sign.
4. IgG, C3 deposition at BM creating smooth line in immunofluorescent analysis.

Causes

Causes of Benign Mucosal Pemphigoid
This disease is characterized by bullous lesions of mucous membranes, especially oral and conjunctival membranes. Eye involvement results in scar formation or fibrous synechiae of palpebral and bulbar conjunctivae, with entropion and blindness occurring in almost a third of untreated cases (Figure 1). Disease onset is usually between 40 and 60 years of patient age, and oral lesions are the first manifestation of disease in two-thirds of cases. There is no racial or ethnic predilection, but the disease is much more common in women than in men (male:female ratio = 1:2).

Individual lesions develop slowly and are usually smaller and less frequent during the early months or years of the disease (Figures 2 & 3). Bullae may, however, become more than 3 cm. in diameter (Figure 4) and may remain intact long enough for the clinician to see them as clear or slightly bluish blisters. While there is seldom a surrounding inflammatory halo, the mucosa in the affected region may be quite diffusely erythematous and at least 10% of patients will have a positive Nikolsky test (creation of a blister by pressure or friction).  A ruptured blister leaves a shallow, mildly tender ulcer bed which heals in 7-10 days. Large or secondarily infected lesions may result in scar formation, but this phenomenon is much less severe than it is with conjunctival involvement.

Diagnosis

Diagnosis of Benign Mucosal Pemphigoid
Consult with your doctor.

Treatment

Treatment of Benign Mucosal Pemphigoid
Oral pemphigoid can often be controlled by topical or systemic corticosteroids or other immunosuppressive agents, particularly cyclophosphamide, but there is no cure for this disease. Lesions occur intermittently and may affect different parts of the oral mucosa at different times. Seldom do blisters occur elsewhere in the upper aerodigestive tract, but patients with laryngeal or esophageal involvement will develop dysphagia or esophageal webs. Ocular involvement eventually becomes manifested in 50-85% of cases and skin involvement is eventually seen in up to 30% of cases. The patient should be followed carefully by an ophthalmologist whether or not conjunctival involvement is seen at the time of oral lesion diagnosis.

Prognosis

Prognosis of Benign Mucosal Pemphigoid
Consult with your doctor.

Prevention

Prevention of Benign Mucosal Pemphigoid
Consult with your doctor.