Medical Care
The goal of treatment in mucous membrane pemphigoid (MMP) (cicatricial pemphigoid) is to suppress extensive blister formation, promote healing, and prevent scarring. The lowest dose of medication to suppress disease activity and to minimize the risk for the patient should be used. This disorder is extremely difficult to treat. Even with optimum control, blisters may continue to develop in some patients. The risks and the benefits of therapy must always be evaluated for each patient.
Wound care of erosions includes daily gentle cleaning or compresses, topical agents to promote wound healing, and biologic dressings. The goals of wound care are to minimize trauma to the surrounding skin, to promote healing, and to diminish scarring.
Increased risk of malignancies has been documented in patients with anti–laminin-332 mucous membrane pemphigoid, especially in the first year of disease; hence, appropriate screening is warranted.
Surgical Care
Surgical intervention may be required to improve functioning or to prevent further morbidity. Such intervention is directed at the sequelae of chronic blistering.
Patients with mucous membrane pemphigoid (MMP) and ocular involvement require ongoing ophthalmologic care. Surgical intervention to ablate ingrown eyelashes prevents further ocular damage. Procedures to release entropion have been successful. Tsubota et al [14] reported the long-term outcome in patients with cicatricial ocular disorders treated with limbal allografts. The transfer of epithelial stem cells restored useful vision in these patients, including several patients with ocular mucous membrane pemphigoid. Care should be taken to control the inflammatory component of the disease before and immediately after surgery because patients with mucous membrane pemphigoid frequently experience flare-ups after surgery.
Patients with upper airway disease may develop respiratory compromise requiring tracheostomy.
Patients with esophageal obstruction may require dilatation procedures.
Consultations
The management of mucous membrane pemphigoid (MMP) requires a coordinated team approach. Specific consultations are dictated by the phenotype of the disease and the target organ or organs involved.
The patient management team typically includes a dermatologist with expertise in this area; an internist to assist with monitoring therapy, adverse effects of medications, and the patient's overall health; an ophthalmologist for ocular disease; an otolaryngologist for upper airway evaluation and management; and a dentist for oral disease.
Additional specialists, such as a gynecologist (vulvar disease), a gastroenterologist (esophageal involvement), and an endocrinologist (prophylaxis of osteoporosis in patients receiving long-term systemic corticosteroids), may be indicated.
Diet
Although no dietary restrictions are necessary, patients with oral disease may benefit from avoiding foods high in acid, such as tomatoes and orange juice, and foods with hard surfaces that may mechanically traumatize the oral epithelium, such as chips, nuts, raw vegetables, and uncut fruit.
Patients on oral prednisone should maintain adequate calcium and vitamin D intake through diet and supplements. The daily calcium requirement in patients with no history of kidney stones is 1.5 g/d, and the daily minimum dose of vitamin D is 800 IU/d.
Activity
Patients are encouraged to lead as normal a life as possible; however, cutaneous and mucosal blisters may be induced by trauma. Contact lenses, dental plates, or bridges may precipitate or exacerbate mucosal disease. Patients may benefit by minimizing activities, such as contact sports, that traumatize the skin and precipitate blistering. Nontraumatic exercises, such as swimming or aquatic exercises, may be beneficial.
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Ocular manifestations of cicatricial pemphigoid (mucous membrane pemphigoid) include symblepharon, demonstrated in this photograph by the tethering of the lower lid to the cornea.
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In a patient with more advanced ocular scarring, note the thickening of the lid margins, shortening of the conjunctival sulcus, and scarring. The eyelashes have been epilated after entropion developed.
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By direct immunofluorescence, a linear band of immunoreactants at the epidermal-dermal junction is demonstrated by using a fluorescein-tagged antibody specific for human immunoglobulin G.
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With advanced disease, ankyloblepharon (a fixed globe) develops.
