Oculomucocutaneous changes as paraneoplastic syndromes

Bullous dermatoses such as erythema exsudativum multiform major (EEMM) and bullous pemphigoid can lead to severe ocular involvement. In rare cases, both diseases develop as paraneoplastic syndromes. The immunopathologic mechanisms are discussed. A 69-year-old woman with non-Hodgkin's lymphoma (NHL) of grade IIIb developed EEMM while under systemic treatment with Fluconazole, Ofloxacin, and/or a combination of sulfamethoxazole and trimethoprim after polychemotherapy. In the eye, conjunctival necrosis with sicca syndrome led to Staphylococcus aureus-induced corneal superinfection, perforation, and consecutive keratoplasty à chaud. The patient died 6 weeks after the first presentation. A 44-year-old man with NHL of grade IVa after polychemotherapy developed a bullous pemphigoid affecting the skin, mucous membranes, and both eyes while under systemic treatment with sulfamethoxazole and trimethoprim. Although the underlying malignancy responded well to chemotherapy, the ocular manifestations of the paraneoplastic systemic syndrome slowed down only on treatment with cyclosporin A but not following therapy with azathioprine and cyclophosphamide. Therapy could not stop cicatrization and keratinization of the conjunctiva and cornea. An occult malignancy should be excluded in acute and chronic oculomucocutaneous syndromes. The prognosis for the eye seems to be poor because of the rapid course and the ineffectiveness of therapy as demonstrated in the present cases.