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Sweet-Syndrom (Akute febrile neutrophile Dermatose)

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Book cover Neue Entwicklungen in der Dermatologie

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Zusammenfassung

Die akute febrile neutrophile Dermatose, kurz Sweet-Syndrom, ist seit 1964 als eigenständiges Krankheitsbild bekannt. Sie zeichnet sich durch typische, schmerzhafte, erhabene Plaques aus, die nach einem kurzen grippalen Prodromalstadium nach einem einwöchigen Intervall unter hohem Fieber und Gelenksbeschwerden vorwiegend an den Extremitäten und im Gesicht auftreten. Sie werden in 95% von einer stark erhöhten Blutsenkung und in 67% von einer Leukocytose begleitet. Histologisch ist das Sweet-Syndrom durch ein auffallendes Ödem im oberen Corium und durch ein leukocytäres Infiltrat mit Leukocy- toklasie gekennzeichnet.

Die Krankheit spricht sehr gut auf systemische Cortisongabe und auf Kaliumjodidtherapie an. Als mögliche Ätiologie wird ein infektallergisches Geschehen angenommen. Differentialdiagnostisch sind klinisch ein Erythema exsudativum multiforme und ein Erythema nodosum auszuschließen. Bei Vorliegen einer Leukämie (7%) ähnelt das Sweet-Syndrom auffallend dem bullösen Pyoderma gangra- enosum, mit dem es auch andere Gemeinsamkeiten aufweist.

Summary

Acute febrile neutrophilic dermatosis, first recognized as a distinct syndrome by Sweet in 1964, is characterized by typical, tender, raised plaques which are located mainly on the face and on the limbs.

The eruption develops after one week of an influenza-like syndrome with high fever and arthritis. An increased sedimentation rate is found in 95% of the cases, a leucocytosis in 67% of the cases.

It responds rapidly to systemic corticosteroid and potassium iodide. The origin seems to be successive to a hypersensitivity reaction. S.S. must be differentiated from erythema multiforme and erythema nodo- sum. S.S. associated with acute leucemia (7%) shows a great similitude with bullous pyoderma gangra- enosum, both diseases having common features.

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  1. Dr. Monika Harms

    Present address: Clinique universitaire de Dermatologie, Hôpiatl cantonal, 1211, Genève 4, Suisse, France

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  1. Dermatologische Klinik, Universitätsspital Genf, Schweiz, Switzerland

    Dr. Monika Harms

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  1. Erlangen, Germany

    O. P. Hornstein

  2. Münster-Hornheide, Germany

    M. Hundeiker

  3. Mannheim, Germany

    J. Schönfeld

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Harms, M. (1987). Sweet-Syndrom (Akute febrile neutrophile Dermatose). In: Hornstein, O.P., Hundeiker, M., Schönfeld, J. (eds) Neue Entwicklungen in der Dermatologie. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-72828-0_12

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