Skip to main content
Log in

Buschke-ollendorff syndrome in a grande multipara: A case report and short review of the literature

  • Case Report
  • Published:
Clinical Rheumatology Aims and scope Submit manuscript

Abstract

A 36-year-old short-statured grande multipara (gravida 10, para 8) with diabetes mellitus and hyperlipidaemia was incidentally found to have Buschke-Ollendorff syndrome (osteopoikilosis and dermatofibrosis lenticularis disseminata). The pelvis and hips, followed by the knees, were the sites mainly affected by the osteopoikilosis. The lumbosacral spine was also affected. She had a single connective tissue naevus on the right thigh. Apart from two abortions, the repeated pregnancies were uneventful and all her deliveries were normal at full-term or nearly full-term. With regard to the relationship between Buschke-Ollendorff syndrome and multiparity, it was apparent that multiparity did not affect the involved weight bearing bony structures, nor did the disseminated osteopoikilosis interfere with the endurance of multiple pregnancies. Buschke-Ollendorff syndrome is known generally to have a benign course, a feature that is illustrated in this case, even when associated with the stresses of multiparity. The report also provides a short and updated review of various clinical aspects of the syndrome and its associations, some of which are of a serious nature.

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

  1. Morrison JGL, Wilson Jones E, MacDonald DM. Juvenile elastoma and osteopoikilosis (the Buschke-Ollendorff syndrome). Br J Dermatol 1977;97:417–422.

    PubMed  Google Scholar 

  2. Schonenberg H. Osteopoikilosis mit dermatofibrosis lenticularis disseminata (Buschke-Ollendorff syndrome) Klin Paed 1975; 187:123–133.

    Google Scholar 

  3. Mckusick VA. Osteopoikilosis. In: Mendelian inheritance in man, 7th edn, Baltimore & London: The John Hopkins University Press: 1986:556.

    Google Scholar 

  4. Benli IT, Akalin S, Boysan E, Mumcu EF, Kis M, Turkoglu D. Epidemiological, clinical and radiological aspects of osteopoikilosis. J Bone Joint Surg [Br] 1992;74:504–506.

    Google Scholar 

  5. Silverman FN. Abnormalities of density of cortical diaphyseal and metaphyseal modeling. In: Silverman FS, Kuhn JP, (editors.) Caffey's paediatric X-ray diagnosis, 9th edn. St Louis: Mosby, 1993:1689–92.

    Google Scholar 

  6. Chigira M, Kato K, Mashio K, Shinozaki T. Symmetry of bone lesions in Osteopoikilosis. Report of 4 cases. Acta Orthop Scand 1991;62:495–6.

    PubMed  Google Scholar 

  7. Roberts NM, Langtry JAA, Branfoot AC, Gleeson J, Staughton RCD. Case report: Osteopoikilosis and Buschke-Ollendorff syndrome. Br J Radiol 1993;66:468–470.

    PubMed  Google Scholar 

  8. Sim E. Osteopoikilosis-Fraktuheilung. Unfallchirurgie 1989; 15:303–5.

    PubMed  Google Scholar 

  9. Ayling RM, Evans PEL. Giant cell tumor in a patient with osteopoikilosis. Acta Orthop Scand 1988;59:74–6.

    PubMed  Google Scholar 

  10. Mindell ER, Northup CS, Douglas HO, Jr. Osteosarcoma associated with osteopoikilosis: Case report. J Bone Joint Surg [Am] 1978;60:406–8.

    Google Scholar 

  11. Grimer RJ, Davies AM, Starkie CM, Sneath RS. Chondrosarcoma in a patient with osteopoikilosis. Rev Chir Orthop 1989;75:188–90.

    PubMed  Google Scholar 

  12. Pacifici R, Murphy WA, Teitelbaum SL, Whyte MP. Mixed sclerosing bone dystrophy: 42-year follow-up of a case reported as osteopetrosis. Calcif-Tissue Int 1986;38:175–85.

    PubMed  Google Scholar 

  13. Llado-Blanche A, Covas-Planells I, Estrach-Planella T. Changes in phosphorus-calcium metabolism in osteopoikilosis (Buschke-Ollendorff syndrome). An Esp Pediatr 1989;31:139–41.

    Google Scholar 

  14. Thieberg MD, Stone MS, Siegfried EC. What syndrome is this? Buschke-Ollendorff syndrome. Pediatr Dermatol 1993;10:86–7.

    Google Scholar 

  15. Ostrowski DM, Gilula LA. Mixed sclerosing bone dystrophy presenting with upper extremity deformities. A case report and review of the literature. J Hand Surg [Br] 1992;17:108–12.

    Google Scholar 

  16. Tong EC, Samii M, Tchang F. Bone imaging as an aid for the diagnosis of osteopoikilosis. Clin Nucl Med 1988;13:816–9.

    PubMed  Google Scholar 

  17. Mungovan JA, Tung GA, Lambiase RE, Noto RB, Davis RP. Tc-99m MDP uptake in osteopoikilosis. Clin Nucl Med 1994;19:6–8.

    PubMed  Google Scholar 

  18. Danielson L, Midtgaard K, Christensen HE. Osteopoikilosis associated dermatofibrosis lenticularis disseminata. Arch Dermatol 1969;100:465–70.

    PubMed  Google Scholar 

  19. Trattner A, David M, Rothem A, Ben-David E, Sandbank M. Buschke-Ollendorff syndrome of the scalp: Histologic and ultrastuctural findings. J Am Acad Dermatol 1991;24:822–4.

    PubMed  Google Scholar 

  20. Uitto J, Shabman A. Heritable skin diseases with molecular defects in collagen and elastin. Dermatol Clin 1987;5:63–84.

    PubMed  Google Scholar 

  21. Verbov J, Graham R. Buschke-Ollendorff syndrome — disseminated dermatofibrosis with osteopoikilosis. Clin Exp Dermatol 1986;11:17–26.

    PubMed  Google Scholar 

  22. Giro MG, Duvic M, Smith LT, Kennedy R, Rapini R, Arnett FC, et al. Buschke-Ollendorff syndrome associated with elevated elastin production by affected skin fibroblasts in culture. J Invest Dermatol 1992;99:129–37.

    PubMed  Google Scholar 

  23. Piette-Brion B, Lowy Motulsky M, Ledoux Corbusier M, Achten G. Dermatofibromas, elastomas and deafness: a new case of the Buschke-Ollendorff syndrome. Dermatologica 1984;168:255–8.

    PubMed  Google Scholar 

  24. Strosberg JM, Alder RG. Otosclerosis associated with osteopoikilosis. JAMA 1981;246:2030–1.

    PubMed  Google Scholar 

  25. Schnur RE, Grace K, Herzberg A. Buschke-Ollendorff syndrome, otosclerosis and congenital spinal stenosis. Pediatr Dermatol 1994;11:31–4.

    PubMed  Google Scholar 

  26. Reinhardt LA, Rountree CB, Wilkin JK. Buschke-Ollendorff syndrome. Cutis 1983;31:94–6.

    PubMed  Google Scholar 

  27. Schorr WF, Opitz JM, Reyes CN. The connective tissue nevus-osteopoikilosis syndrome. Arch Dermatol 1972;106:208–14.

    PubMed  Google Scholar 

  28. Raque CJ, Wood MG. Connective tissue nevus: dermatofibrosis lenticularis disseminata. Arch Dermatol 1970;102:390–6.

    PubMed  Google Scholar 

  29. Gunal I, Seber S, Basaran N, Artan S, Gunal K, Gokturk E. Dacryocystitis associated with osteopoikilosis. Clin Genet 1993;44:211–3.

    PubMed  Google Scholar 

  30. De la Salmoniere P, Janier M, Chemlal K, Lazareth I, Carlotti A, Charasson I, et al. Buschke-Ollendorff syndrome. Ann Dermatol Venerol 1994;121:718–20.

    Google Scholar 

  31. Hauck RM, Manders EK. Familial syndromes with skin tumor markers. Ann Plast Surg 1994;33:102–11.

    PubMed  Google Scholar 

  32. Kobus RJ, Lubbres LM, Coleman CR. Connective tissue nevus and osteopoikilosis in the hand: the Buschke-Ollendorff syndrome. J Hand Surg [Am] 1989;14:535–8.

    Google Scholar 

  33. Walpole IR, Manners PJ. Clinical considerations in Buschke-Ollendorff syndrome. Clin Genet 1990;37:59–63.

    PubMed  Google Scholar 

  34. Megele R, Stosiek N, Kriegel W, Krizinger T, Dranter E. A case of osteopoikilosis combined with dermal changes and compression syndromes of peripheral nerves. Z Orthop Ihre Grenzegeb 1991;129:465–8.

    Google Scholar 

  35. Colla F, Bruhlmann P, Panizzon R, Michel BA. Osteopoikilosis-skin and joint manifestations. Z Rheumatol 1995;54:123–7.

    PubMed  Google Scholar 

  36. Beazly JM. Special circumstances affecting labour. In: Whitfield CR (ed), Dewhurst's textbook of obstetric and gynaecology for postgraduates. Blackwell Science, 1995:315–6.

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Al Attia, H.M., Sherif, A.M. Buschke-ollendorff syndrome in a grande multipara: A case report and short review of the literature. Clin Rheumatol 17, 172–175 (1998). https://doi.org/10.1007/BF01452270

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01452270

Keywords

Navigation