Cicatricial Pemphigoid

Cicatricial Pemphigoid (CP) is a distinct disease entity of unknown but presumed autoimmune cause. Its effect on the eyes is characterized by chronic cicatrizing conjunctivitis, progressive conjunctival subepithelial fibrosis, and resultant fornix foreshortening, symblepharon formation, trichiasis, distichiasis, meibomian duct obstruction, and eventual lacrimal duct compromise with reduced tear supply to the ocular surface. It is of substantial ophthalmologic interest because of its bilaterality, and because it eventually results in legal blindness due to corneal scarring in a high proportion of people with the disease. No topical therapy is effective in stopping the progression of scarring of the conjunctiva. High doses of systemic prednisone can control the process, but the long-term consequences of moderate-to-high doses of systemic steroid required for control of CP are unacceptable. Systemic immunosuppressive therapy, alone or in combination with systemic dapsone, is highly effective in controlling the disease, and may cure it in many instances.These clinical characteristics coupled with the ocular and the systemic immunopathologic correlates in patients with CP show it to be a systemic disease, and most evidence strongly suggests it is caused or propagated by a defect in immunoregulation.CP has been one of the main interests of Dr. Foster's research group. Struck by the catastrophic consequences of this disease, the total lack of efficacy of conventional therapy, and the circumstantial evidence suggesting that CP is an autoimmune disorder, they made a major commitment to the care and study of patients with CP. The studies have been both clinical and basic, and the care has involved personal responsibility not only for the ocular therapy but also for the systemic management and monitoring of the patients.The purpose of this book is to describe the results of their experience with 130 patients who have CP.