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Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis

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Abstract

In 1983 the Proteus syndrome was delineated by Wiedemann et al. [12]. We report a 10-month-old girl, a further child affected by the new syndrome. The typical signs are macrodactyly, hemihypertrophy, pigmented nevi, hyperkeratosis, and subcutaneous hamartomatous tumours. Our patient shows an aggressive lipomatosis on the trunk and local relapses after surgical interventions in the regions involved. Histology of the adipose tissue showed considerable anisocytosis and increased cell volume.

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References

  1. 1.

    Aldenhoff P, Kunze J, Kaufmann HJ (1982) “True macrodactyly” und Lungenfehlbildungen. In: Tolksdorf M, Spranger J (eds) Klinische Genetik der Pädiatrie, 3. Symposium in Kiel 1981. Milupa, Friedrichsdorf, pp 299–307

  2. 2.

    Anderson WAD (1952) Synopsis pathology. CV Mosby, St. Louis

  3. 3.

    Cohen MM Jr (1981) Overgrowth syndromes. In: Shafie MEL, Klippel CH (eds) Associated congenital anomalies. Williams and Wilkins, Baltimore

  4. 4.

    Cohen MM Jr (1982) The child with multiple birth defects. Raven Press, New York

  5. 5.

    Cordier MP, Robert JM, Malek R (1982) Un cas de magalosyndactylie. J Genet Hum 30:393–401

  6. 6.

    Greulich WW, Pyle SI (1966) Radiographic atlas of skeletal development of hand and wrist, 2nd edn. Stanford University Press, California

  7. 7.

    Higginbottom MC, Schultz P (1982) The Bannayan syndrome: An autosomal dominant disorder consisting of macrocephaly, lipomas, hemangiomas, and risk for intracranial tumors. Pediatrics 69:632–634

  8. 8.

    Lachmann RS, Finkelstein J, Mehringer CM, Maenza R (1983) Congenital aggressive lipomatosis. Skeletal Radiol 9:248–254

  9. 9.

    Mücke J, Martin I, Bührdel P, Sandig K-R (1978) Das Exomphalos-Makroglassie-Gigantismus-syndrom. Dtsch Gesundh-Wesen 33:2332–2337

  10. 10.

    Opitz JM, Herrmann J (1977) The study of genetic diseases and malformations. Birth Defects. Alan R Liss, New York XIII 6, pp 45–66

  11. 11.

    Wiedemann H-R (1983) Personal communication

  12. 12.

    Wiedemann H-R, Burgio GR, Aldenhoff P, Kunze J, Kaufmann HJ, Schirg E (1983) The Proteus syndrome. Eur J Pediatr 140:5–12

  13. 13.

    Wiedemann H-R, Grosse F-R, Dibbern H (1982) Das charakteristische Syndrom. Blickdiagnose von Syndromen, 2nd edn. Schattauer, Stuttgart New-York, pp 330–331

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Correspondence to J. Mücke.

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Mücke, J., Willgerodt, H., Künzel, R. et al. Variability in the Proteus syndrome: report of an affected child with progressive lipomatosis. Eur J Pediatr 143, 320–323 (1985). https://doi.org/10.1007/BF00442313

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Key words

  • Proteus syndrome
  • Aggressive lipomatosis