Comprehensive diagnosis and treatment of cicatricial ectropion caused by pemphigus vulgaris

Pemphigus vulgaris is a rare autoimmune disorder that arises from autoantibodies targeting antigens in intraepidermal cell cohesion, leading to painful erosions and flaccid blisters. Given that pemphigus vulgaris can induce cicatricial ectropion, a comprehensive diagnosis and treatment is essential. A 61-year-old man was referred from a dermatovenereology clinic with a two-month history of watery eyes and a downward-pulled right inferior palpebra. The initial manifestation involved bullae across the body, including the periocular skin and oral mucosa. The visual acuity (VA) in his right eye was 5/6, and in his left eye, it was 5/12. The examination of his right eye revealed ectropion, an everted inferior punctum, madarosis, 1 mm lagophthalmos, scleral show, and ectropion that did not resolve during the 2-finger test, with a lid distraction of 8 mm based on the distraction test. Despite blinking during the snap-back test, his inferior palpebra did not return to its normal position. The diagnosis of cicatricial ectropion caused by pemphigus vulgaris was made. Cicatricial ectropion is the outward rotation of the eyelid margin due to anterior lamella shortening from scar tissue. This shortening leads to the eyelid margin moving away from the eyeball, causing watery eyes, irritation, and cosmetic concerns. Lubrication treatment for the patient's eyes included chloramphenicol eye ointment every eight hours and artificial tears (1 drop every 4 hours) to avoid exposure of the eyeball. The treatment administered in the dermatovenereology clinic consisted of prednisone, natrium fusidate ointment, vaselin album, clobetasol propionate 0.05% cream, and mefenamic acid. Scar tissue in the anterior lamella caused by chronic inflammation of periorbital pemphigus vulgaris could result in ectropion. The management aimed to protect the eyeball from exposure and treat the underlying disease.