Infantile Digital Fibromatosis

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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, as is shown in the image below. Reye first described infantile digital fibromatosis in 1965 as a recurring digital fibrous tumor. ^[1]

The etiology of infantile digital fibromatosis is unknown. Defective organization of actin filaments in myofibroblasts has been hypothesized. 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. ^{[2, 3]}

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

Males and females are equally affected by infantile digital fibromatosis.

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.

The prognosis for infantile digital fibromatosis is excellent. Infantile digital fibromatosis is benign, without evidence of malignant transformation or metastases. There may be single or multiple nodules. 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). Recurrence is common after excision.

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

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. 2009 Jan. 33(1):1-13. [Medline].

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

Kaya A, Yuca SA, Karaman K, Erten R, Dogan M, Bektas MS, et al. Infantile digital fibromatosis (inclusion body fibromatosis) observed in a baby without finger involvement. Indian J Dermatol. 2013 Mar. 58(2):160. [Medline]. [Full Text].

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

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

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

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

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

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

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

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

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

Monique Gupta Kumar, MD, MPhil Assistant Professor of Dermatology, Emory University School of Medicine

Monique Gupta Kumar, MD, MPhil is a member of the following medical societies: American Academy of Dermatology, International Society of Dermatology, Pediatric Dermatology Research Alliance, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Susan Joy Bayliss, MD Director of Pediatric Dermatology, Departments of Internal Medicine and Pediatrics, St Louis Children’s Hospital; Professor, Washington University in St Louis School of Medicine

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

Disclosure: Nothing to disclose.

Michael J Wells, MD, FAAD Dermatologic/Mohs Surgeon, The Surgery Center at Plano Dermatology

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

Disclosure: Nothing to disclose.

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.

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

Disclosure: Nothing to disclose.

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.

Infantile Digital Fibromatosis

Research & References of Infantile Digital Fibromatosis|A&C Accounting And Tax Services
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