Surgical Care

Surgical excision of the dermal lesions is indicated only for cosmetic reasons. In some patients, surgical treatment of deafness is possible. Surgical treatment of associated conditions, such as stenosis of the aortae, should be performed as appropriate.

Consultations

Note the following potential consultations:

Laryngologic examination with audiogram
Ophthalmologic examination with ophthalmoscopy
General physical examination with ECG

Activity

Osteopoikilosis is asymptomatic; however, 15-20% of patients experience pain and joint effusions. Usually, no special restrictions in activity are required.^[5]

Long-Term Monitoring

There are no specific follow-up requirements in the case of asymptomatic dermatofibrosis lenticularis (Buschke-Ollendorff syndrome).

Benli IT, Akalin S, Boysan E, Mumcu EF, Kis M, Türkoglu D. Epidemiological, clinical and radiological aspects of osteopoikilosis. J Bone Joint Surg Br. 1992 Jul. 74(4):504-6. [Medline].
Crivellato E. Disseminated nevus anelasticus. Int J Dermatol. 1986 Apr. 25(3):171-3. [Medline].
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].
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. 2005 Feb 1. 14(3):437-45. [Medline].
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].
Umaretiya PJ, Miest RY, Tollefson MM. A 5-year-old with connective tissue nevi: Buschke-Ollendorff syndrome. J Pediatr. 2014 Jul. 165(1):206. [Medline].
Boyaci A, Boyaci N, Tutoglu A. Familial osteopoikilosis. J Back Musculoskelet Rehabil. 2013. 26(3):247-50. [Medline].
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 Aug. 99(2):129-37. [Medline].
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. 2003 Dec. 49(6):1163-6. [Medline].
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. 2004 Nov. 36(11):1213-8. [Medline].
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. 1991 May. 24(5 Pt 2):822-4. [Medline].
Ben-Asher E, Zelzer E, Lancet D. LEMD3: the gene responsible for bone density disorders (osteopoikilosis). Isr Med Assoc J. 2005 Apr. 7(4):273-4. [Medline].
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].
Lagier R, Mbakop A, Bigler A. Osteopoikilosis: a radiological and pathological study. Skeletal Radiol. 1984. 11(3):161-8. [Medline].
Saussine A, Marrou K, Delanoé P, et al. Connective tissue nevi: An entity revisited. J Am Acad Dermatol. 2011 Oct 18. [Medline].
Pope V, Dupuis L, Kannu P, Mendoza-Londono R, Sajic D, So J, et al. Buschke-Ollendorff syndrome: a novel case series and systematic review. Br J Dermatol. 2016 Apr. 174 (4):723-9. [Medline].
Blum AE, Hatter A, Honda K, Lu K. An unusual presentation of Buschke-Ollendorff syndrome. J Am Acad Dermatol. 2011 Jul. 65(1):e25-6. [Medline].
Ayling RM, Evans PE. Giant cell tumor in a patient with osteopoikilosis. Acta Orthop Scand. 1988 Feb. 59(1):74-6. [Medline].
Morrison JG, Jones EW, MacDonald DM. Juvenile elastoma and osteopoikilosis (the Buschke--Ollendorff syndrome). Br J Dermatol. 1977 Oct. 97(4):417-22. [Medline].
Schorr WF, Optiz JM, Reyes CN. The connective tissue nevus-osteopoikilosis syndrome. Arch Dermatol. 1972 Aug. 106(2):208-14. [Medline].
Gracia-Cazaña T, Frias M, Roselló R, Vera-Álvarez J, Gilaberte Y. PASH syndrome associated with osteopoikilosis. Int J Dermatol. 2015 Sep. 54 (9):e369-71. [Medline].
Al Attia HM, Sherif AM. Buschke-Ollendorff syndrome in a grande multipara: a case report and short review of the literature. Clin Rheumatol. 1998. 17(2):172-5. [Medline].
Atherton DJ, Wells RS. Juvenile elastoma and osteopoikilosis (the Buschke-Ollendorf syndrome). Clin Exp Dermatol. 1982 Jan. 7(1):109-13. [Medline].
Cantatore FP, Carrozzo M, Loperfido MC. Mixed sclerosing bone dystrophy with features resembling osteopoikilosis and osteopathia striata. Clin Rheumatol. 1991 Jun. 10(2):191-5. [Medline].
Miklaszewska M, Szybejko-Machaj G, Szepietowski J. [Buschke-Ollendorf syndrome - case report with a literature review.]. Dermatol Klin Zabieg. 1999. 2:85-9.
Schnur RE, Grace K, Herzberg A. Buschke-Ollendorff syndrome, otosclerosis, and congenital spinal stenosis. Pediatr Dermatol. 1994 Mar. 11(1):31-4. [Medline].
Woodrow SL, Pope FM, Handfield-Jones SE. The Buschke-Ollendorff syndrome presenting as familial elastic tissue naevi. Br J Dermatol. 2001 Apr. 144(4):890-3. [Medline].
Surrenti T, Callea F, De Horatio LT, Diociaiuti A, El Hachem M. Buschke-Ollendorff syndrome: sparing unnecessary investigations. Cutis. 2014 Aug. 94(2):97-100. [Medline].
Cañueto J, Román C, Santos-Briz Á, Ciria S, González R, Unamuno P. Papular elastorrhexis and Buschke-Ollendorff syndrome are different entities. J Am Acad Dermatol. 2011 Jul. 65 (1):e7-9. [Medline].
Kennedy JG, Donahue JR, Aydin H, Hoang BH, Huvos A, Morris C. Metastatic breast carcinoma to bone disguised by osteopoikilosis. Skeletal Radiol. 2003 Apr. 32(4):240-3. [Medline].
Holbrook KA, Byers PH. Structural abnormalities in the dermal collagen and elastic matrix from the skin of patients with inherited connective tissue disorders. J Invest Dermatol. 1982 Jul. 79 Suppl 1:7s-16s. [Medline].

Media Gallery

Plaque of grouped papular eruptions on the thigh.
Hematoxylin and eosin stain shows the histopathologic features of the skin lesions.
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 histopathologic features of the skin lesions.
Weigert stain of the elastic fibers shows the histopathologic features of the skin lesions.
Plaque of grouped papules on the abdominal coat.

of 6

Author

Vernon J Forrester, MD Resident Physician, Department of Dermatology, UVA Health System, University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Krista Roncone University of Virginia School of Medicine

Disclosure: Nothing to disclose.

Barbara B Wilson, MD Edward P Cawley Associate Professor, Department of Dermatology, University of Virginia School of Medicine

Barbara B Wilson, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Medical Society of Virginia, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Former Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, Association of Military Dermatologists, Phi Beta Kappa

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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: Received consulting fee from Orfagen for consulting; Received consulting fee from Maruho for consulting; Received consulting fee from Astellas for consulting; Received consulting fee from Abbott for consulting; Received consulting fee from Leo Pharma for consulting; Received consulting fee from Biogenoma for consulting; Received honoraria from Janssen for speaking and teaching; Received honoraria from Medac for speaking and teaching; Received consulting fee from Dignity Sciences for consulting; .

Grazyna Szybejko-Machaj, MD, PhD Assistant Professor, Department of Dermatology, Wroclaw Medical University

Disclosure: Nothing to disclose.

Lukasz Matusiak, MD, PhD Assistant, Department and Clinic of Dermatology, Venereology and Allergology, Medical University of Wroclaw

Disclosure: Nothing to disclose.

Acknowledgements

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.

Mieczyslawa Miklaszewska, MD, PhD Professor, Department of Dermatology, Medical University in Wroclaw, Poland

Disclosure: Nothing to disclose.

Sections Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome)
Overview
Presentation
DDx
Workup
Treatment
Media Gallery
References

Dermatofibrosis Lenticularis (Buschke-Ollendorf Syndrome) Treatment & Management

Surgical Care

Consultations

Activity

Long-Term Monitoring

References

Tables

Contributor Information and Disclosures

What would you like to print?