Cicatricial Pemphigoid Has Numerous Manifestations

by Dr. Joyce Rico

Cicatricial pemphigoid is an autoimmune blistering disease that predominately effects mucous membranes, including the mouth and throat, eyes, and genitalia. Patients with skin involvement present with tense blisters or erosion, often on the head and neck, or at sites of trauma. Blisters heal with scarring, which can lead to vision loss or blindness, hoarseness or airway obstruction. Patients with other autoimmune blistering diseases may also have mucosal involvement.

There are wide differences in clinical manifestation of this disease, with some patients presenting with eye disease and others presenting with nose, mouth and throat involvement. Most patients with CP have onset in the seventh decade. CP has been rarely diagnosed in children under age 10.

CP is an autoimmune blistering disease associated with autoantibodies directed against normal skin antigens present at the junction of epidermis and dermis.

The two major antigens associated with CP are collagen XVII and epiligrin (laminin 5). There is no clinical differences between patients with anti-collagen XVII and anti-epiligrin reactivity. Determination of the specific antigen that a patient is reacting against is not readily available, except in specialized research laboratories, but will not change the patient’s treatment.

Cicatricial pemphigoid has been treated with many different therapies including topical agents, intralesional steroids, antiinflammatory agents such as Dapsone and Prednisone, and immunosup-presants such as azathioprine (Imuran), cyclophosphamide (Cytoxan), cyclosporin (Neoral), mycophen-olate mofetil (CellCept). Intravenous immune globulin (IVIG) has been used for patients with eye disease. Treatment of this condition requires a coordinated approach by the patients primary care physician, and specialists such as ophthalmologists, gynecologists, otolaryngologists (ENT), and dermatologists.

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