Histology: Subepidermal blistering that heals with scar formation is the hallmark of this disease. The blistering takes place just below the keratinocyte, within in the lamina lucida. Immunohistochemical staining with collagen type IV shows that the blister plane is above the level of the lamina densa. The immunostaining and routine hematoxylin and eosin staining show a picture very similar to that of bullous pemphigoid. Linear immunoglobulin G and complement C3 immunofluorescent staining is present along the basement membrane zone.
Pathogenesis: Autoantibody formation against proteins of the basement membrane zone has been linked to cicatricial pemphigoid. Many different antibodies against these proteins exist, including antibodies against the laminins, bullous pemphigoid antigens 180 and 230, and many other proteins as yet unclassified. The heterogeneity in antibody production likely accounts for the varying clinical phenotypes that are expressed.
Treatment: Prednisone is the drug used to treat the disease initially. After the disease is under some control, the addition of a steroid-sparring immunosuppressant should be attempted. Commonly used medications include azathioprine, methotrexate, mycophenolate mofetil, and cyclophosphamide. Dapsone and sulfapyridine, a similar medication that can be used in place of dapsone, have had some success treating this disease. Intravenous immunoglobulin (IVIG) has been used with success in refractory cases.
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