Cicatricial pemphigoid: Diagnosis and treatment

Cicatricial pemphigoid (CP) is a systemic autoimmune disease with both ocular and nonocular manifestations. CP, unlike bullous pemphigoid, produces scarring of the affected skin [1, 2]. It can lead to catastrophic ophthalmic consequences, such as bilateral blindness due to corneal scarring. CP also can be fatal when it produces strictures from scarring in the esophagus or in the trachea. Histopathological and immunopathological studies indicate a possible defect in immunoregulation [1]. It is critically important that the diagnosis be made early, with conjunctival biopsy and immunohistochemistry. Progression of the disease can be halted only through systemic chemotherapeutic strategies. Reconstructive surgery can be performed only after all inflammatory activities have been controlled with chemotherapy.