Pure ocular cicatricial pemphigoid - A distinct immunopathologic subset ofcicatricial pemphigoid

Objective: To determine whether ocular cicatricial pemphigoid (OCP) may rep resent a distinct immunopathologic disease when it is pure ocular cicatrici al pemphigoid (POCP) (e.g., only confined to the conjunctiva) or when it is associated with skin or extraocular mucous membrane lesions or both (OCP+) . Design: Prospective, immunologic, and immunopathologic study with special e mphasis on direct immunoelectron microscopy, Participants: Six patients with POCP and seven patients with OCP+. Intervention: After informed consent was obtained, a conjunctival biopsy wa s performed in all patients. Skin and extraocular mucosa biopsy specimens w ere harvested in selected cases only. Main Outcome Measures: Results of direct immunofluorescence and direct immunoelectron microscopy w ithout freezing on conjunctival and skin biopsy specimens, indirect immunof luorescence, and Western immunoblotting analysis were analyzed. Results: Re sults of direct immunoelectron microscopic examination of the conjunctiva s howed the presence of immune deposits in the upper lamina lucida of the bas ement membrane zone in the six patients with POCP, whereas the immune react ants were located in the lower part of the lamina lucida and in the lamina densa of the basement membrane zone (conjunctiva, buccal mucosa, and skin) in the seven patients with OCP+. Direct immunofluorescence was positive in the biopsy specimens of three patients with POCP (50%) and the seven patien ts with OCP+ (100%), Results of indirect immunofluorescence study showed ci rculating autoantibody levels only in two patients with OCP+, and results o f Western immunoblot analysis were negative. Conclusions: Results of direct immunoelectron microscopic examination of th e conjunctiva support the hypothesis that POCP may be a disease entity dist inct from mucocutaneous cicatricial pemphigoid.