Clinical Information

Laminin 332 (LM332) pemphigoid is a rare subepithelial autoimmune blistering disease that can affect the conjunctival, esophageal, oral, and genital mucosa and skin. With LM332 pemphigoid, there is a risk of blindness and esophageal stricture, among other serious complications. In addition, approximately 20% to 30% of patients with LM332 pemphigoid have an underlying malignancy driving their mucocutaneous disease. Therefore, it is generally accepted that patients found to have circulating LM332 antibodies should be screened for an occult malignancy.

P200 pemphigoid, a rare subepithelial autoimmune blistering disease affecting the skin, shares some clinical characteristics with psoriasis, a much more common inflammatory dermatosis of the skin. However, identification of circulating p200 autoantibodies predicts the development of blisters and portends a worse clinical therapeutic response.

Collagen VII autoantibodies are pathogenic in two rare subepithelial autoimmune blistering diseases: epidermolysis bullosa acquisita (EBA) and bullous systemic lupus erythematosus (BSLE). A diagnosis of EBA is confirmed upon identification of circulating autoantibodies to collagen VII and predicts a refractory treatment course. In addition, patients with EBA have a high rate of associated inflammatory bowel disease (IBD), so identification of collagen VII autoantibodies may prompt clinicians to increase surveillance for IBD. In the appropriate clinical context, circulating autoantibodies to collagen VII may support a diagnosis of BSLE. Accurate identification of BSLE is important, as most patients with this condition have severe manifestations of lupus in other organs, such as lupus nephritis. While our immunodermatology laboratory offers another test to detect collagen VII autoantibodies (COL7 / Anti-Collagen type VII, IgG antibodies, Serum), collagen VII is a large protein, rendering autoantibodies against this target immunologically heterogeneous.