Dermatology pp 861-868 | Cite as

Systemic Lipid Storage Diseases

  • Otto Braun-Falco
  • Gerd Plewig
  • Helmut H. Wolff
  • Richard K. Winkelmann

Abstract

In these diseases, which are also called thesaurismoses, there is no increase in serum lipids or lipoproteins as is characteristic of hyperlipoproteinemia. The morphological anomaly is characterized by a generalized proliferation of histiocytoid cells in which lipids are deposited only as a secondary consequence of a genetically determined disorder of cell metabolism. There is usually no possibility of etiotropic treatment of these diseases.

Synonyms

Heredopathia atactica polyneuritiform phytanic acid thesaurismosis. Analphaproteinemaia. Fabry’s disease Angiokeratoma corporis diffusum universale Thesaurismosis hereditaria lipoidica (Ruiter-Pompen-Wyers) Fabry’s syndrome. Constitutional granular gigantism. Cerebroside lipidosis Kerasin lipid histiocytosis. Sphingomyelin lipidosis Sphinhomyelinosis Niemann-Pick syndrome. Farber’s disease familial lipogranulomatosis. Constitutional granular gigantism. 

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Copyright information

© Springer-Verlag Berlin Heidelberg 1991

Authors and Affiliations

  • Otto Braun-Falco
    • 1
  • Gerd Plewig
    • 2
  • Helmut H. Wolff
    • 3
  • Richard K. Winkelmann
    • 4
  1. 1.Dermatologische Klinik und PoliklinikLudwig-Maximilians-Universität MünchenMünchen 2Federal Republic of Germany
  2. 2.Hautklinik der Heinrich-Heine-UniversitätDüsseldorfFederal Republic of Germany
  3. 3.Klinik für Dermatologie und VenerologieMedizinischen Universität zu LübeckLübeck 1Federal Republic of Germany
  4. 4.Dermatology — Dermatopathology, Mayo ClinicMayo Medical SchoolRochesterUSA

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