eMedicine Specialties > Dermatology > Pediatric Diseases

Maffucci Syndrome: Follow-up

Author: Raymond T Kuwahara, MD, Dermatologist, Private Practice
Coauthor(s): Ron Rasberry, MD, Chief of Dermatology, Veterans Medical Center at Memphis; Associate Professor, Department of Dermatology, University of Tennessee at Memphis
Contributor Information and Disclosures

Updated: Nov 12, 2008

Follow-up

Further Inpatient Care

  • No inpatient care is needed for this syndrome, unless complications arise from the hemangiomas (bleeding) or malignant transformation of the skin and skeletal lesions.

Further Outpatient Care

  • As discussed earlier, regular physical examinations are required to evaluate new or changing skin and bone abnormalities.

Complications

  • Neoplastic changes occur in enchondromas. Chondrosarcoma is the most common neoplasm in this syndrome, affecting about 30% of patients (see Pathophysiology).
  • Enchondromas can cause a fracture, leading to further complications, such as shortened or unequal length limbs.

Prognosis

  • Patients with Maffucci syndrome usually lead reasonably normal lives with a normal life expectancy if no malignant transformation occurs.
  • Although the skeletal malformations can sometimes be crippling, patients have managed to perform activities of daily living rather well.

Patient Education

 


More on Maffucci Syndrome

Overview: Maffucci Syndrome
Differential Diagnoses & Workup: Maffucci Syndrome
Treatment & Medication: Maffucci Syndrome
Follow-up: Maffucci Syndrome
Multimedia: Maffucci Syndrome
References
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References

  1. Albregts AE, Rapini RP. Malignancy in Maffucci's syndrome. Dermatol Clin. Jan 1995;13(1):73-8. [Medline].

  2. Kaplan RP, Wang JT, Amron DM, Kaplan L. Maffucci's syndrome: two case reports with a literature review. J Am Acad Dermatol. Nov 1993;29(5 Pt 2):894-9. [Medline].

  3. Faik A, Allali F, El Hassani S, Hajjaj-Hassouni N. Maffucci's syndrome: a case report. Clin Rheumatol. Feb 2006;25(1):88-91. [Medline].

  4. Spitz JL. Maffucci syndrome. In: Genodermatosis, A Clinical Guide to Genetic Skin Disorders. Baltimore, Md: Lippincott Williams & Wilkins; 2005:118-9.

  5. Tilsley DA, Burden PW. A case of Maffucci's syndrome. Br J Dermatol. Sep 1981;105(3):331-6. [Medline].

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Further Reading

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Keywords

dyschondrodysplasia with hemangiomas, enchondromatosis with multiple cavernous hemangiomas, multiple angiomas and endochondromas

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Contributor Information and Disclosures

Author

Raymond T Kuwahara, MD, Dermatologist, Private Practice
Raymond T Kuwahara, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Ron Rasberry, MD, Chief of Dermatology, Veterans Medical Center at Memphis; Associate Professor, Department of Dermatology, University of Tennessee at Memphis
Ron Rasberry, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, Arkansas Medical Society, Association of Military Surgeons of the US, Royal Society of Medicine, and Southern Medical Association
Disclosure: Nothing to disclose.

Medical Editor

Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France
Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology and American Dermatological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory
Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System
William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology
Disclosure: elsevier Royalty Other; american college of physicians Honoraria Other

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