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Sinushistiozytose mit massiver Lymphadenopathie (Rosai-Dorfman-Erkrankung)

Sinushistiocytosis with massive lymphadenopathy (Rosai-Dorfman disease)

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Zusammenfassung

Die Sinushistiozytose mit massiver Lymphadenopathie (SHML) ist eine seltene, schmerzlose, lymphoproliferative Erkrankung unbekannter Ursache mit meist benignem Verlauf. Etwa 40% der Patienten weisen einen extranodalen Befall auf, wobei die Haut mit 27% am häufigsten befallen ist. Die folgende Kasuistik beschreibt eine sehr ausgedehnte Erkrankung mit Befall des Respirationstraktes, der Nieren sowie der Haut. Histologisch zeigte sich das charakteristische Bild der SHML mit Emperipolesis sowie immunhistochemischer Positivität der Histiozyten auf S100 und zahlreiche Makrophagenantigene.

Abstract

Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare, painless lymphoproliferative disorder of unknown origin with a usually benign course. About 40% of the patients show an extranodal involvement with skin being the most common site in 27% of these patients. We describe a patient with widespread disease involving the respiratory tract, kidneys and skin. Histopathology revealed the characteristic features of SHML with emperipolesis and immunohistochemical positivity of histiocytes for S100 and macrophage-associated antigens.

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Authors and Affiliations

  1. Hautklinik, Klinikum Kassel, Mönchebergstraße 41–43, 34125, Kassel, Deutschland

    M.R. Becker & R. Rompel

  2. Pathologie, Klinikum Kassel, Kassel, Deutschland

    T. Gaiser & P. Middel

Authors
  1. M.R. Becker
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  2. T. Gaiser
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  3. R. Rompel
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  4. P. Middel
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Correspondence to M.R. Becker.

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Becker, M., Gaiser, T., Rompel, R. et al. Sinushistiozytose mit massiver Lymphadenopathie (Rosai-Dorfman-Erkrankung). Hautarzt 59, 400–403 (2008). https://doi.org/10.1007/s00105-007-1385-y

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  • DOI: https://doi.org/10.1007/s00105-007-1385-y

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