GRAFT-VERSUS-HOST-LIKE MUCOCUTANEOUS ERUPTIONS WITH SEROLOGICAL FEATURES OF PARANEOPLASTIC PEMPHIGUS AND SYSTEMIC LUPUS-ERYTHEMATOSUS IN A PATIENT WITH NON-HODGKINS-LYMPHOMA

A 63-year-old male patient spontaneously developed severe erosive orog enital mucositis, palmoplantar and gluteal inflammatory lesions resist ant to therapy, The skin lesions clinically and histologically resembl ed lichen-planus-like graft-versus-host disease. Investigation for an underlying autoimmune or malignant disorder revealed a centrocytic-cen troblastic low-grade non-Hodgkin's lymphoma (according to the Kiel cla ssification) in the bone marrow, mesenterial and iliacal lymphoma. Ser ological titers were intermittently positive for ANA, anti-Sm/U1RNP, a nti-Re and anti-dsDNA. Immunoprecipitation of lysates from radiolabele d human keratinocytes with the patient's serum revealed circulating an tibodies against 210-kD (desmoplakin II), 190- and 170-kD antigens but none against the 230-kD antigen or 250-kD desmoplakin I. Under cytost atic chemotherapy the lymphomas showed complete and longlasting remiss ion, whereas the mucocutaneous lesions persisted. Six years after diag nosis? the mucocutaneous lesions are sufficiently controlled by immuno suppressive therapy. In the presented case, several features of lympho ma-associated dysimmunoreactivity are assumed that bring about the int rinsic production of various autoantibodies typical of paraneoplastic pemphigus and systemic lupus erythematosus.