Bullous pemphigoid (BP) is a blistering disorder of the skin and mucosa that may coexist with
inflammatory bowel disease (IBD). The authors' experiences with peristomal and generalized BP in five patients (three with
ulcerative colitis [UC] post
colostomy surgery and two with
Crohn's disease [CD] post
ileostomy surgery, time since surgery 5 to 20 years) is described. The patients presented with peristomal
blisters and erosions, subsequently confirmed as BP by skin biopsy. Treatments for the
skin disease included potent alcohol-based topical
corticosteroids, oral
tetracyclines, and oral
corticosteroids. In three patients (two with UC, one with CD), the initially localized peristomal disease later became generalized across the skin; these patients were more likely to require systemic immunosuppressive therapy. Because an involvement of
plectin, a cytoskeletal
protein that attaches skin and mucosal cells to their extracellular matrix, in IBD has been shown, it is possible that this
protein forms the missing link between IBD and BP via
epitope spreading. The
inflammation of IBD exposes
plectin, stimulating a secondary immune response that may, in susceptible individuals, crossreact with the skin, provoking BP. Further research into this area could enable clinical testing for
plectin auto-
antibodies in patient sera, possibly preempting the development of BP and expediting the initiation of early effective treatment.