eMedicine Specialties > Dermatology > Pediatric Diseases

Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome): Multimedia

Author: Lukasz Matusiak, MD, Assistant, Department and Clinic of Dermatology, Venereology and Allergology, Medical University of Wroclaw
Coauthor(s): Grazyna Szybejko-Machaj, MD, PhD, Assistant Professor, Department of Dermatology, Wroclaw Medical University; Jacek C Szepietowski, MD, PhD, Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland
Contributor Information and Disclosures

Updated: Jul 2, 2008

Multimedia

Plaque of grouped papular eruptions on the thigh.Media file 1: Plaque of grouped papular eruptions on the thigh.
Plaque of grouped papular eruptions on the thigh.

Plaque of grouped papular eruptions on the thigh.

Hematoxylin and eosin stain shows the histopathol...Media file 2: Hematoxylin and eosin stain shows the histopathologic features of the skin lesions.
Hematoxylin and eosin stain shows the histopathol...

Hematoxylin and eosin stain shows the histopathologic features of the skin lesions.

Small longitudinal lesions of increased bone dens...Media file 3: Small longitudinal lesions of increased bone density in the proximal epiphysis of the left tibial bone and in the distal epiphysis of the right tibial bone.
Small longitudinal lesions of increased bone dens...

Small longitudinal lesions of increased bone density in the proximal epiphysis of the left tibial bone and in the distal epiphysis of the right tibial bone.

Orcein stain of elastic fibers shows the histopat...Media file 4: Orcein stain of elastic fibers shows the histopathologic features of the skin lesions.
Orcein stain of elastic fibers shows the histopat...

Orcein stain of elastic fibers shows the histopathologic features of the skin lesions.

Weigert stain of the elastic fibers shows the his...Media file 5: Weigert stain of the elastic fibers shows the histopathologic features of the skin lesions.
Weigert stain of the elastic fibers shows the his...

Weigert stain of the elastic fibers shows the histopathologic features of the skin lesions.

Plaque of grouped papules on the abdominal coat.Media file 6: Plaque of grouped papules on the abdominal coat.
Plaque of grouped papules on the abdominal coat.

Plaque of grouped papules on the abdominal coat.

More on Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)

Overview: Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
Differential Diagnoses & Workup: Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
Treatment & Medication: Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
Follow-up: Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
Multimedia: Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
References

References

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  2. Crivellato E. Disseminated nevus anelasticus. Int J Dermatol. Apr 1986;25(3):171-3. [Medline].

  3. Dahan S, Bonafe JL, Laroche M, et al. [Iconography of Buschke Ollendorff syndrome: x ray computed tomography and nuclear magnetic resonance of osteopoikilosis]. Ann Dermatol Venereol. 1989;116(3):225-30. [Medline].

  4. Lin F, Morrison JM, Wu W, Worman HJ. MAN1, an integral protein of the inner nuclear membrane, binds Smad2 and Smad3 and antagonizes transforming growth factor-beta signaling. Hum Mol Genet. Feb 1 2005;14(3):437-45. [Medline].

  5. de la Salmonière P, Janier M, Chemlal K, Lazareth I, Carlotti A, Charasson I, et al. [Buschke-Ollendorff syndrome]. Ann Dermatol Venereol. 1994;121(10):718-20. [Medline].

  6. Ehrig T, Cockerell CJ. Buschke-Ollendorff syndrome: report of a case and interpretation of the clinical phenotype as a type 2 segmental manifestation of an autosomal dominant skin disease. J Am Acad Dermatol. Dec 2003;49(6):1163-6. [Medline].

  7. Hellemans J, Preobrazhenska O, Willaert A, Debeer P, Verdonk PC, Costa T, et al. Loss-of-function mutations in LEMD3 result in osteopoikilosis, Buschke-Ollendorff syndrome and melorheostosis. Nat Genet. Nov 2004;36(11):1213-8. [Medline].

  8. Trattner A, David M, Rothem A, Ben-David E, Sandbank M. Buschke-Ollendorff syndrome of the scalp: histologic and ultrastructural findings. J Am Acad Dermatol. May 1991;24(5 Pt 2):822-4. [Medline].

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  10. Grimer RJ, Davies AM, Starkie CM, Sneath RS. [Chondrosarcoma in a patient with osteopoikilosis. Apropos of a case]. Rev Chir Orthop Reparatrice Appar Mot. 1989;75(3):188-90. [Medline].

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  13. 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. Aug 1992;99(2):129-37. [Medline].

  14. Morrison JG, Jones EW, MacDonald DM. Juvenile elastoma and osteopoikilosis (the Buschke--Ollendorff syndrome). Br J Dermatol. Oct 1977;97(4):417-22. [Medline].

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  18. Cantatore FP, Carrozzo M, Loperfido MC. Mixed sclerosing bone dystrophy with features resembling osteopoikilosis and osteopathia striata. Clin Rheumatol. Jun 1991;10(2):191-5. [Medline].

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  25. Uitto J, Santa Cruz DJ, Starcher BC, Whyte MP, Murphy WA. Biochemical and ultrastructural demonstration of elastin accumulation in the skin lesions of the Buschke-Ollendorff syndrome. J Invest Dermatol. Apr 1981;76(4):284-7. [Medline].

Further Reading

Keywords

disseminated lenticular dermatofibrosis, osteopoikilosis, dermatofibrosis lenticularis disseminata with osteopoikilosis, ectodermal dysplasia of connective tissue, osteodermatopoikilosis, osteopathia condensans disseminata

Contributor Information and Disclosures

Author

Lukasz Matusiak, MD, Assistant, Department and Clinic of Dermatology, Venereology and Allergology, Medical University of Wroclaw
Disclosure: Nothing to disclose.

Coauthor(s)

Grazyna Szybejko-Machaj, MD, PhD, Assistant Professor, Department of Dermatology, Wroclaw Medical University
Disclosure: Nothing to disclose.

Jacek C Szepietowski, MD, PhD, Professor, Vice-Head, Department of Dermatology, Venereology and Allergology, Wroclaw Medical University; Director of the Institute of Immunology and Experimental Therapy, Polish Academy of Sciences, Poland
Disclosure: Stiefel Salary Employment

Medical Editor

Mark A Crowe, MD, Assistant Clinical Instructor, Department of Medicine, Division of Dermatology, University of Washington School of Medicine
Mark A Crowe, MD is a member of the following medical societies: American Academy of Dermatology and North American Clinical Dermatologic Society
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

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