Graft-versus-host-like mucocutaneous eruptions with serological features of paraneoplastic pemphigus and systemic lupus erythematosus in a patient with non-Hodgkin's lymphoma

V. Mahler, C. Antoni, G. J. Anhalt, H. J. Koch, K. P. Peters, B. Manger, J. R. Kalden, O. P. Harnstein

Research output: Contribution to journalArticlepeer-review

14 Scopus citations

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 mucocutaneaus 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.

Original languageEnglish (US)
Pages (from-to)78-83
Number of pages6
JournalDermatology
Volume197
Issue number1
DOIs
StatePublished - 1998

Keywords

  • Graft-versus-host-like mucocutaneous disease
  • Lichen ruber
  • Non-Hodgkin's lymphoma
  • Overlap syndrome
  • Paraneoplastic pemphigus
  • Systemic lupus erythematosus

ASJC Scopus subject areas

  • Dermatology

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