Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and systemic lupus erythematosus in a patient with non-Hodgkin's lymphoma.
A 63-year-old male patient spontaneously developed severe erosive orogenital mucositis, palmoplantar and gluteal inflammatory lesions resistant to therapy. The skin lesions clinically and histologically resembled lichen-planus-like graft-versus-host disease. Investigation for an underlying autoimmune or malignant disorder revealed a centrocytic-centroblastic low-grade non-Hodgkin's lymphoma (according to the Kiel classification) in the bone marrow, mesenterial and iliacal lymphoma. Serological titers were intermittently positive for ANA, anti-Sm/U1RNP, anti-Ro and anti-dsDNA. Immunoprecipitation of lysates from radiolabeled human keratinocytes with the patient's serum revealed circulating antibodies against 210-kD (desmoplakin II), 190- and 170-kD antigens but none against the 230-kD antigen or 250-kD desmoplakin I. Under cytostatic chemotherapy the lymphomas showed complete and long-lasting remission, whereas the mucocutaneous lesions persisted. Six years after diagnosis, the mucocutaneous lesions are sufficiently controlled by immunosuppressive therapy. In the presented case, several features of lymphoma-associated dysimmunoreactivity are assumed that bring about the intrinsic production of various autoantibodies typical of paraneoplastic pemphigus and systemic lupus erythematosus.
Department of Dermatology, University of Erlangen-Nuremberg, Germany.
V
V
Mahler
C
C
Antoni
G J
GJ
Anhalt
H J
HJ
Koch
K P
KP
Peters
B
B
Manger
J R
JR
Kalden
O P
OP
Hornstein
D003937:Diagnosis, Differential; D005455:Fluorescent Antibody Technique; D006086:Graft vs Host Disease / Q000209:etiology*; D006229:Hand Dermatoses; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000276:immunology*; D008228:Lymphoma, Non-Hodgkin / Q000150:complications / Q000175:diagnosis* / Q000276:immunology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009061:Mouth Mucosa; D010257:Paraneoplastic Syndromes / Q000150:complications / Q000276:immunology*; D010392:Pemphigus / Q000150:complications / Q000276:immunology*