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Juvenile hyaline Fibromatose

Juvenile hyaline fibromatosis

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Zusammenfassung

Die juvenile hyaline Fibromatose ist eine sehr seltene autosomal-rezessiv vererbte Bindegewebserkrankung. Wir berichten über einen derzeit 6-jährigen Jungen, bei dem sich bei normaler geistiger Entwicklung seit dem 2. Lebensjahr zunehmend kleinknotige Hautveränderungen im Bereich der Ohrmuscheln und der Nasenflügel ausbilden, ferner größere derbe Knoten im Bereich des Capillitiums, des Rückens und der Metaphalangen sowie eine ausgeprägte Gingivahypertrophie.

Abstract

Juvenile hyaline fibromatosis is a rare autosomal recessive disease of the connective tissue. We present the case of a 6-year-old normal mental developed boy with confluent pearly papules behind the ears and in the paranasal folds, firm nodules of the scalp, the back and metaphalangs, and severe gingival hypertrophy.

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Authors and Affiliations

  1. Klinik für Dermatologie, Allergologie und Umweltmedizin, Katholisches Klinikum Duisburg, Barbarastraße 67, 47167, Duisburg, Deutschland

    K. Güldner, J. Schaller & J. Kunze

  2. Dermatohistologisches Labor, Duisburg, Deutschland

    C. Hendricks & J. Schaller

Authors
  1. K. Güldner
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  2. C. Hendricks
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  3. J. Schaller
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  4. J. Kunze
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Corresponding author

Correspondence to K. Güldner.

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Güldner, K., Hendricks, C., Schaller, J. et al. Juvenile hyaline Fibromatose. Hautarzt 60, 740–742 (2009). https://doi.org/10.1007/s00105-008-1698-5

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  • DOI: https://doi.org/10.1007/s00105-008-1698-5

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