Infantile Digital Fibromatosis 

  • Author: Theresa Schroeder Devere, MD; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Jan 12, 2012
 

Background

Infantile digital fibromatosis is a benign asymptomatic nodular proliferation of fibrous tissue occurring almost exclusively on the dorsal and lateral aspects of the fingers or the toes. Reye first described infantile digital fibromatosis in 1965 as a recurring digital fibrous tumor.[1]

Firm, nontender, erythematous nodule on the fifth Firm, nontender, erythematous nodule on the fifth finger of a 17-month-old boy.
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Pathophysiology

The etiology of infantile digital fibromatosis is unknown. It has been suggested that possible deregulation of the normal bone morphogenetic protein (a member of the transforming growth factor-β superfamily) mediated apoptotic pathway may explain the location of these lesions at the sites of digital septation. Transforming growth factor-β1 also mediates myofibroblast differentiation from fibroblasts. Myofibroblasts are the primary cell type in this disorder.[2, 3]

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Epidemiology

Frequency

International

Infantile digital fibromatosis is rare, with approximately 250 cases reported worldwide.

Mortality/Morbidity

Infantile digital fibromatosis is benign, without evidence of malignant transformation or metastases. Nodules are often multiple, and recurrence is common after excision. Infantile digital fibromatosis lesions tend to spontaneously involute without scarring. Rarely, the lesions can cause functional impairment or deformity. Rare cases of ulceration have been reported. The infantile digital fibromatosis lesions tend to grow slowly in the first month, then rapidly grow over about a year, followed by spontaneous resolution over 1-10 years (average 2-3 y).

Sex

Males and females are equally affected by infantile digital fibromatosis.

Age

Most nodules appear in the first few months of life; one third are congenital, and 75-80% are noted during the first year of life. Reports of infantile digital fibromatosis developing in older children and adults are rare.

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Contributor Information and Disclosures
Author

Theresa Schroeder Devere, MD  Assistant Professor of Dermatology, Residency Director, Oregon Health Sciences University

Theresa Schroeder Devere, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Coauthor(s)

Susan Bayliss Mallory, MD  Director of Pediatric Dermatology, Departments of Internal Medicine and Pediatrics, Professor, Saint Louis Children's Hospital, Washington University

Susan Bayliss Mallory, MD is a member of the following medical societies: American Academy of Dermatology, American Academy of Pediatrics, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Van Perry, MD  Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas School of Medicine at San Antonio

Van Perry, MD is a member of the following medical societies: American Academy of Dermatology and American Society for Laser Medicine and Surgery

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

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

Disclosure: Nothing to disclose.

References

  1. Reye RD. Recrring Digital Fibrous tumors of childhood. Arch Pathol. Sep 1965;80:228-31. [Medline].

  2. Laskin WB, Miettinen M, Fetsch JF. Infantile digital fibroma/fibromatosis: a clinicopathologic and immunohistochemical study of 69 tumors from 57 patients with long-term follow-up. Am J Surg Pathol. Jan 2009;33(1):1-13. [Medline].

  3. Taylor HO, Gellis SE, Schmidt BA, Upton J, Rogers GF. Infantile digital fibromatosis. Ann Plast Surg. Oct 2008;61(4):472-6. [Medline].

  4. Choi KC, Hashimoto K, Setoyama M, Kagetsu N, Tronnier M, Sturman S. Infantile digital fibromatosis. Immunohistochemical and immunoelectron microscopic studies. J Cutan Pathol. Aug 1990;17(4):225-32. [Medline].

  5. Ishii N, Matsui K, Ichiyama S, et al. A case of infantile digital fibromatosis showing spontaneous regression.. Br J Dermatol. Jul 1989;121(1):129-33. [Medline].

  6. Kawaguchi M, Mitsuhashi Y, Hozumi Y, Kondo S. A case of infantile digital fibromatosis with spontaneous regression. J Dermatol. Aug 1998;25(8):523-6. [Medline].

  7. Niamba P, Leaute-Labreze C, Boralevi F, et al. Further documentation of spontaneous regression of infantile digital fibromatosis. Pediatr Dermatol. May-Jun 2007;24(3):280-4. [Medline].

  8. Spingardi O, Zoccolan A, Venturino E. Infantile digital fibromatosis: our experience and long-term results. Chir Main. Feb 2011;30(1):62-5. [Medline].

  9. Albertini JG, Welsch MJ, Conger LA, Libow LF, Elston DM. Infantile digital fibroma treated with mohs micrographic surgery. Dermatol Surg. Oct 2002;28(10):959-61. [Medline].

  10. Oh CK, Son HS, Kwon YW, Jang HS, Kwon KS. Intralesional fluorouracil injection in infantile digital fibromatosis. Arch Dermatol. May 2005;141(5):549-50. [Medline].

  11. Holmes WJ, Mishra A, McArthur P. Intra-lesional steroid for the management of symptomatic Infantile Digital Fibromatosis. J Plast Reconstr Aesthet Surg. May 2011;64(5):632-7. [Medline].

  12. Azam SH, Nicholas JL. Recurring infantile digital fibromatosis: report of two cases. J Pediatr Surg. Jan 1995;30(1):89-90. [Medline].

  13. Dabney KW, MacEwen GD, Davis NE. Recurring digital fibrous tumor of childhood: case report with long-term follow-up and review of the literature. J Pediatr Orthop. Sep-Oct 1986;6(5):612-7. [Medline].

  14. O'Gorman DJ. Infantile digital fibromatosis. Proc R Soc Med. Sep 1974;67(9):880. [Medline].

 
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Dermal tumor with interlacing spindle-shaped cells and collagen bundles. Perinuclear eosinophilic inclusion bodies are not visible at this magnification.
Firm, nontender, erythematous nodule on the fifth finger of a 17-month-old boy.
A dermal nodule extending into the subcutaneous fat.
 
 
 
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