OCULOMUCOCUTANEOUS CHANGES AS PARANEOPLASTIC SYNDROMES

Bullous dermatoses such as erythema exsudativum multiforme major (EEMM ) and bullous pemphigoid can lead to severe ocular involvement. In rar e cases, both diseases develop as paraneoplastic syndromes. The immuno pathologic mechanisms are discussed. A 69-year-old woman with non-Hodg kin's lymphoma (NHL) of grade IIIb developed EEMM while under systemic treatment with Fluconazole, Ofloxacin, and/or a combination of sulfam ethoxazole and trimethoprim after polychemotherapy. In the eye, conjun ctival necrosis with sicca syndrome led to Staphylococcus aureus-induc ed corneal superinfection, perforation, and consecutive keratoplasty a chaud. The patient died 6 weeks after the first presentation. A 44-ye ar-old man with NHL of grade IVa after polychemotherapy developed a bu llous pemphigoid affecting the skin, mucous membranes, and both eyes w hile 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 wi th azathioprine and cyclophosphamide. Therapy could not stop cicatriza tion and keratinization of the conjunctiva and cornea. An occult malig nancy should be excluded in acute and chronic oculomucocutaneous syndr omes. The prognosis for the eye seems to be poor because of the rapid course and the ineffectiveness of therapy as demonstrated in the prese nt cases.