Summary
Massive membranocystic lesions of the lung were found in an autopsy case of Nasu-Hakola disease. The membranocystic structures are virtually identical to those found in adipose tissue, including bone marrow. Capillary endothelia and alveolar epithelial lining cells do not participate in membranous structure formation, these structures being found in the alveolar septa and not apparently of an embolic nature. They may be related to alveolar septal cells in a broad sense. The pathogenesis of this disease is discussed in relation to the lesions of the lung.
Similar content being viewed by others
References
Ham AW (1965) The structure of interalveolar walls or septa, pp. 758-766. Histology 5th eds. Lippincott Company (1965)
Hakola P (1972) Lipomembranous polycystic osteodysplasia. Acta psychiat. scand. Suppl. 232:1–172
Harada H (1975) Ein Fall von “membranöser Lipodystrophie (Nasu)”, unter besonderer Berücksichtigung des psychiatrischen und neuropathologischen Befundes. Folia Psychiat Neurol Jpn 29:169–177
Jarvi OH, Hakola P, Sourander P (1970) A new entity of phacomatosis; a bone lesion (hereditary angionecrotic polycystic osteodysplasia). Acta Pathol Microbiol Scand Suppl 251:27
Matsushita M, Oyanagi S, Hanawa S, Shiraki H, Kosaka K, Nasu-Hakola disease (membranous lipodystrophy). A case report. Acta Neuropathol (Belr) 54:89–93
Nasu T, Tsukahara Y, Terayama K, Mamiya N (1970) An autopsy case of membranous lipodystrophy demonstrating myelo-osteopathy of various long bones and leucodystrophy demonstrating myelo-osteopathy of various long bones and leucodystrophy of the brain. Record of various long bones and leucodystrophy of the brain. Record of the 59th Tokyo Path. Conf. pp. 10–13 (in Japanese)
Nasu T, Tsukahara Y, Terayama K (1973) A lipid metabolic disease- “membranous lipodystrophy” - An autopsy case demonstrating numerous peculiar membrane structures composed of compound lipid in bone and bone marrow and various adipose tissues. Acta Pathol Jpn 23:539–558
Tanaka J (1980) Leucoencephalopathic alteration in membranous lipodystrophy. Acta neuropathol (Berl) 50:193–197
Tashiro Y, Koide N, Watanabe Y, Muraya M, Satomi K (1976) Membranous lipodystrophy (Nasu) as a metabolic disorder of lipid, special reference to morphological observation. Clin Orthop Surg. (Jpn) 11:614–625
Yagishita S, Ito Y, Ikezaki R (1976) Lipomembranous polycystic osteodysplasia. Virchows Arch [Pathol Anat] 372:245–251
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Yagishita, S., ITo, Y., Sakai, H. et al. Membranocystic lesions of the lung in Nasu-Hakola disease. Vichows Archiv A Pathol Anat 408, 211–217 (1985). https://doi.org/10.1007/BF00707983
Accepted:
Issue Date:
DOI: https://doi.org/10.1007/BF00707983