Klinische Wochenschrift

, Volume 66, Issue 8, pp 365–372 | Cite as

Weber-christian panniculitis with systemic cytophagic histiocytosis

  • H. Steininger
  • M. Missmahl
Kasuistik

Summary

A 49-year-old woman suffered from Weber-Christian panniculitis with a typically periodic course. Subsequently, the attacks of the disease developed to a severe state: high fever, endotoxinemia, pancytopenia, and clotting disorder, in addition to the cutaneous manifestations. One and a half years after the outbreak of the disease the patient died in a septic shock. At autopsy all three stages of Weber-Christian panniculitis were found. In addition, an immense proliferation of benign cytophagic histiocytes could be observed in the bone marrow, spleen, lymph nodes and, less distinctly, in the fatty and interstitial tissues of the visceral organs. As a nosologic entity, the Weber-Christian disease is frequently questioned. Some authors consider the lobular, histiocytic, cytophagic panniculitis a unique syndrome. On the other hand, it must be emphasized that benign, cytophagic histiocytosis may exist associated with infections or may accompany different diseases. Furthermore, the Weber-Christian panniculitis is clinically and morphologically well defined.

Key words

Weber-Christian panniculitis Systemic cytophagic histiocytosis Pancytopenia Clotting disorder Nosologic entity 

Abbreviations

PAP method

peroxydase-anti-peroxydase method

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Copyright information

© Springer-Verlag 1988

Authors and Affiliations

  • H. Steininger
    • 1
  • M. Missmahl
    • 2
  1. 1.Pathologisches Institut der Universität Erlangen-NürnbergGermany
  2. 2.Institut für Klinische Immunologie und RheumatologieUniversität Erlangen-NürnbergGermany

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