You are in: eMedicine Specialties > Dermatology > BENIGN NEOPLASMS Infantile Digital FibromatosisArticle Last Updated: Mar 28, 2007AUTHOR AND EDITOR INFORMATIONSection 1 of 10
  Author: Theresa L Schroeder Devere, MD, Assistant Professor of Dermatology, Associate Residency Director, Oregon Health Sciences University Theresa L Schroeder Devere is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and Phi Beta Kappa Coauthor(s): Susan Bayliss Mallory, MD, Director of Pediatric Dermatology, Departments of Internal Medicine and Pediatrics, Professor, Saint Louis Children's Hospital, Washington University Editors: Jean Paul Ortonne, MD, Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France; Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center; Van Perry, MD, Assistant Professor, Department of Medicine, Division of Dermatology, University of Texas Health Science Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center Author and Editor Disclosure Synonyms and related keywords: IDF, Reye tumor, recurring digital fibroma of childhood, recurring digital fibrous tumor INTRODUCTIONSection 2 of 10
  BackgroundInfantile digital fibromatosis (IDF) 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 it in 1965 as a recurring digital fibrous tumor. PathophysiologyThe etiology of this condition is unknown. Although a viral etiology was initially suspected because of cytoplasmic inclusion bodies found on histologic examination, subsequent electron microscopy and polymerase chain reaction findings do not support a viral cause. FrequencyInternationalIDF is rare, with approximately 100 cases reported worldwide. Mortality/MorbidityIDF is benign without evidence of malignant transformation or metastases. Nodules are often multiple, and recurrence is common after excision. Spontaneous involution without scarring has been reported in approximately 12% of patients. Rarely do the lesions cause functional impairment or deformity, but they have become ulcerated in some instances. SexMales and females are equally affected. AgeMost 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 IDF developing in older children and adults are rare. CLINICALSection 3 of 10
HistoryPatients are asymptomatic, without associated systemic symptoms. Physical
CausesThe cause is unknown. DIFFERENTIALSSection 4 of 10
Acquired Digital Fibrokeratoma Dermatofibroma Granuloma Annulare Keloid and Hypertrophic Scar Knuckle Pads Multicentric Reticulohistiocytosis Neurilemoma Sarcoidosis Xanthomas
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| Drug Name | Triamcinolone (Aristocort) |
|---|---|
| Description | For inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Can be injected intralesionally. |
| Adult Dose | 10 mg/mL intralesional |
| Pediatric Dose | 10 mg/mL intralesional; total of 0.1-0.3 mL/lesion, depending on the size of the lesion; repeat q4-6wk until lesions show adequate regression |
| Contraindications | Documented hypersensitivity; fungal, viral, and bacterial skin infections |
| Interactions | Coadministration with barbiturates, phenytoin, and rifampin decreases effects |
| Pregnancy | C - Safety for use during pregnancy has not been established. |
| Precautions | Multiple complications (eg, severe infections, hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression) may occur; abrupt discontinuation of glucocorticoids may cause adrenal crisis |
These agents inhibit cell growth and proliferation.
| Drug Name | Fluorouracil (Efudex, Adrucil, Fluoroplex) |
|---|---|
| Description | Pyrimidine analogue shown to inhibit dermal fibroblast proliferation and collagen synthesis in cell culture. |
| Adult Dose | 50 mg/mL intralesionally (total of 10 mg [0.2 mL]) per lesion monthly until lesion regresses |
| Pediatric Dose | Administer as in adults |
| Contraindications | Documented hypersensitivity; poor nutritional state, bone marrow suppression, pregnancy, severe renal function impairment, chicken pox, or herpes zoster; dihydropyrimidine dehydrogenase enzyme deficiency (topical route) |
| Interactions | None reported with local injection |
| Pregnancy | D - Unsafe in pregnancy |
| Precautions | Common adverse effects include pain, purpura, ulceration, burning sensation, and pigmentary disturbance at injection site |
| Media file 1: Dermal tumor with interlacing spindle-shaped cells and collagen bundles. Perinuclear eosinophilic inclusion bodies are not visible at this magnification. | |
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| Media file 2: Firm, nontender, erythematous nodule on the fifth finger of a 17-month-old boy. | |
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| Media file 3: A dermal nodule extending into the subcutaneous fat. | |
 | View Full Size Image | Media type: Photo |
Infantile Digital Fibromatosis excerpt
Article Last Updated: Mar 28, 2007
