[Severe eye involvement in pemphigus vulgaris].

Pemphigus vulgaris (PV) is an immune-mediated vesiculobullous disease of the skin and mucous membranes. Generally, patients with PV present first with oral lesions, which may precede the cutaneous lesions, such as bullae and erosions, by several months. An ocular manifestation is unusual. The most common ophthalmologic involvement in pemphigus is conjunctivitis, but without progressive scarring such as occurs in ocular cictricial pemphigoid. Corneal involvement is very rare. CASE REPORT. We report on severe ocular involvement in a 56-year-old male Turkish patient with PV. Ophthalmologic findings included conjunctival cicatrization, corneal ulceration and corneal perforation, despite immunosuppressive therapy with azathioprine and corticosteroids. A penetrating keratoplasty was performed, but rapidly failed because of corneal vascularization. CONCLUSION. If a chronic bullous dermatosis leads to severe eye involvement, PV should be considered in the differential diagnosis besides ocular cicatricial pemphigoid. Pemphigus vulgaris can be differentiated on the basis of clinical findings and histopathological and immunohistological features. Generally, PV can be treated with steroids or with a combination of an immunosuppressant and steroids. However, the combination of prednisone and azathioprine could not prevent corneal perforation in our patient.