Abstract |
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.
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Authors | V Mahler, C Antoni, G J Anhalt, H J Koch, K P Peters, B Manger, J R Kalden, O P Hornstein |
Journal | Dermatology (Basel, Switzerland)
(Dermatology)
Vol. 197
Issue 1
Pg. 78-83
( 1998)
ISSN: 1018-8665 [Print] Switzerland |
PMID | 9693195
(Publication Type: Case Reports, Journal Article, Review)
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Topics |
- Diagnosis, Differential
- Fluorescent Antibody Technique
- Graft vs Host Disease
(etiology)
- Hand Dermatoses
- Humans
- Lupus Erythematosus, Systemic
(complications, immunology)
- Lymphoma, Non-Hodgkin
(complications, diagnosis, immunology, pathology)
- Male
- Middle Aged
- Mouth Mucosa
- Paraneoplastic Syndromes
(complications, immunology)
- Pemphigus
(complications, immunology)
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