Skip to main content
Log in

Lipomembranous polycystic osteodysplasia

  • Case Reports
  • Published:
Virchows Archiv A Aims and scope Submit manuscript

Summary

A case of lipomembranous polycystic Osteodysplasia is presented. The clinical features were characterized by multiple cystic changes of the bones and progressive psychomotor retardation. Although the diagnosis of polyostotic fibrous dysplasia of bone was made first because of expanded tumorlike lesions in bones, pathology of bone and bone marrow confirmed the diagnosis of lipomembranous polycystic Osteodysplasia.

Light microscopy showed a great number of peculiar undulating membranous structures in the adipose tissue. With the electron microscope the membrane seemed to be composed of an accumulation of endoplasmic reticulum-like tubular profiles. They appear first, in close connection with fat droplets, within the mesenchymal cell.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  • Abul-Haj, S.K., Martz, D.G., Douglass, W.F., Geppert, L.J.: Farber's disease; Report of a case with observations on its histogenesis and notes on the nature of the stored material. J. Pediat. 61, 221–232 (1962)

    Google Scholar 

  • Farber, S.: A lipid metabolic disorder-disseminated “lipogranulomatosis”—A syndrome with similarity to, and important difference from, Niemann-Pick and Hand-Schular-Christian disease. Amer. J. Dis. Child. 84, 499–500 (1952)

    Google Scholar 

  • Hakola, P.: Lipomembranous polycystic osteodysplasia. Acta psychiat. scand., Suppl. 232, 1–172 (1972)

    Google Scholar 

  • Hakola, P., Jarvi, O.H., Sourander, P.: Osteodystrophia polycystica hereditaria combined with sclerosing leucoencephalopathy. A new entity of dementia praesenilis group. Acta neurol. scand., Suppl. 43, 78–79 (1970)

    Google Scholar 

  • Jarvi, O.H., Jakola, P.: A new entity of phacomatosis; a bone lesion (hereditary angionecrotic polycystic osteodysplasia). Acta path. microbiol. scand., Suppl. 251, 27 (1970)

    Google Scholar 

  • Jarvi, O.H., Lauttamys, L.L., Solonen, K.A.: Cystic capillary-necrotic osteodysplasia. A systemic bone disease probably caused by arteriolar and capillary necrosis. Relation to brain affections. Abstracts. Seventh Internat. Congress of Internat. Academy of Pathology, 291–292 (1968)

  • Kashima, H., Kasahara, T., Hara, Y., Kimura, S., Maejo, S., Yakumaru, K.: A case of membranous lipodystrophy (Nasu). Jap. Acad. Clin. Neurol., Abstracts, 67 (1973)

  • Nasu, T., Tsukahara, Y., Terayama, K.: A lipid metabolic disease—“membranous lipodystrophy” — an autopsy case demonstrating peculiar membrane-structures composed of compound lipid in bone and bone marrow and various adipose tissues. Acta path. jap. 23, 539–558 (1973)

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Yagishita, S., Ito, Y. & Ikezaki, R. Lipomembranous polycystic osteodysplasia. Virchows Arch. A Path. Anat. and Histol. 372, 245–251 (1976). https://doi.org/10.1007/BF00433283

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00433283

Key words

Navigation