eMedicine Specialties > Dermatology > Benign Neoplasms

Infantile Digital Fibromatosis

Author: Theresa Schroeder Devere, MD, Assistant Professor of Dermatology, Residency Director, Oregon Health Sciences University
Coauthor(s): Susan Bayliss Mallory, MD, Director of Pediatric Dermatology, Departments of Internal Medicine and Pediatrics, Professor, Saint Louis Children's Hospital, Washington University
Contributor Information and Disclosures

Updated: Oct 30, 2009

Introduction

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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Firm, nontender, erythematous nodule on the fifth...

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


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

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.

Clinical

History

Patients with infantile digital fibromatosis are asymptomatic, without associated systemic symptoms.

Physical

Single or multiple, firm, erythematous, dermal nodules with a smooth dome-shaped surface appear on the dorsolateral aspect of the distal phalanges of the digits. Infantile digital fibromatosis lesions can grow up to 2 cm in diameter, yet they rarely cause functional impairment or deformity. The distribution of lesions includes the fingers and the toes with equal frequency, except for sparing of the thumbs and the great toes. Rare extradigital sites reported include the hands, the feet, the arms, the nose, the breasts, and the tongue.

Causes

The cause of infantile digital fibromatosis is unknown.

More on Infantile Digital Fibromatosis

Overview: Infantile Digital Fibromatosis
Differential Diagnoses & Workup: Infantile Digital Fibromatosis
Treatment & Medication: Infantile Digital Fibromatosis
Follow-up: Infantile Digital Fibromatosis
Multimedia: Infantile Digital Fibromatosis
References
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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. 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].

  9. 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].

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

  11. 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].

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

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Further Reading

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Keywords

infantile digital fibromatosis, IDF, Reye tumor, recurring digital fibroma of childhood, recurring digital fibrous tumor, inclusion body fibromatosis

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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.

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

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
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.

Managing Editor

Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center
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.

CME Editor

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, 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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