Unrecognized cicatricial pemphigoid with oral manifestations and ocular complications. A case report

Abstract

Cicatricial pemphigoid is an autoimmune bullous disease characterized by mucous membrane fibrosis with resulting scarring, predominantly in the conjunctival and oral mucosa, which rarely involves skin changes. The majority of patients present with painful erosions or desquamative scarring gingivitis, resulting in eating and drinking disorders. Typical ocular lesions include chronic scarring conjunctivitis with progressive subconjunctival fibrosis, fornix foreshortening and synechia formation between the bulbar and palpebral conjunctiva, occasionally resulting in blindness. A 69-year-old woman was admitted to our Department for intense pain and severe burning sensation in the oral cavity, induced by several erosions and solitary blisters, lasting for 3 years. She was also diagnosed with the right eye symblepharon, lid entropion, trichiasis, leukoma and blindness of the right eye. The diagnosis of cicatricial pemphigoid was based on clinical picture and histopathology combined with immunofluorescence methods, with therapy initiated thereupon. Systemic corticosteroid (methylprednisolone) therapy in combination with azathioprine proved successful in the treatment of oral lesions as well as for stabilization of ocular lesions. Unfortunately, the patient was diagnosed in the advanced stage when scarring had already occurred. Prompt recognition of cicatricial pemphigoid and close patient monitoring are an imperative for the future prognosis of the disease.