Cicatricial pemphigoid (Benign mucosal pemphigoid)
Which are the symptoms?
In bullous pemphigoid, there is often a prodromal phase, characterized by itchy inflammatory plaques that resemble eczema or urticaria. Subsequently, a bullous phase occurs, in which bullous, tense, dome-shaped formations with a diameter of about 1–3 cm develop, which subsequently turn into erosion and crusts at the sites of rupture of the bubbles. Lesions usually heal without scarring. The limbs and body are most commonly affected. In 10-30% of cases, there is concomitant damage to the mucous membranes.
On the other hand, mucosal pemphigoid usually occurs as desquamative gingivitis or erosions in the oral mucosa. Other potential sites include the conjunctiva of the eye, skin, pharynx, genital mucosa, nasal mucosa, and esophagus. Unlike bullous pemphigoid, pemphigoid lesions of the mucous membranes tend to disappear with scarring. Scars can cause serious functional changes in the affected organs. A particularly severe form is ocular cicatricial pemphigoid, burdened with a high risk of irreversible visual impairment.
How is it diagnosed?
How is it treated?
Therapy is based on the use of local or systemic corticosteroids in cases of widespread disease. It is often necessary to resort to systemic immunosuppressive therapy (methotrexate, mycophenolate mofetil, azathioprine, cyclophosphamide, rituximab) under the supervision of a specialist. Eye involvement with this disease should be closely monitored by an experienced ophthalmologist.