Ocular pemphigoid

Cicatricial pemphigoid, bullous pemphigoid, and pemphigus are bullous diseases of the skin and mucous membranes that may be associated with ocular involvement. Of the three, cicatricial pemphigoid is the bullous disease that not only involves the eye most often but also causes the most severe ocular disease. Whereas bullous pemphigoid rarely involves the eye, pemphigus may be associated with conjunctivitis but rarely causes conjunctival scarring. 1 On the other hand, cicatricial pemphigoid causes progressive conjunctival shrinkage leading to blindness. The distributions of lesions in published series of patients with cicatricial pemphigoid are not equivalent in the ophthalmologic and dermatologic literature. In an analysis of 261 patients with cicatricial pemphigoid from four different dermatologic series, cutaneous lesions were present in 24%, oral lesions were found in 91%, and conjunctival lesions in 66% of patients. 2 Patients with skin and mucous membrane involvement excluding the conjunctiva may not be examined by ophthalmologists and will not be reported in ophthalmologic series. On the other hand, patients in ophthalmic reports all have ocular involvement with or even without involvement of the skin and other mucous membranes. In the largest ophthalmic series consisting of 78 patients, 21% had skin involvement and 50% had oral mucosal involvement. 3 One hundred percent had ocular involvement, which was a prerequisite for referral and inclusion in the study. In the same series, the ages at initial examination ranged from 43–86 years, with a mean of 69 years. The disease affected more women than men, with a ratio of 1.6:1.