Information on Infantile Digital Fibromatosis

Sep 30
09:01

2008

Juliet Cohen

Juliet Cohen

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Infantile Digital fibromas may be associated with facial pigmentary dysplasia, focal dermal hypoplasia, metacarpal and metatarsal disorganization, and limb malformations.

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Infantile 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. This lesion has also been referred to as Reye tumor,Information on Infantile Digital Fibromatosis Articles infantile digital fibroma and recurrent digital fibrous tumour of childhood. Tumors of this type usually occur in infants or young children.  Males and females are equally affected.

These lesions generally present as single or multiple nodules involving the extensor surfaces, most commonly the dorsal and lateral aspect of the distal or middle phalanx. Thumbs and great toes are usually spared. Multiple lesions are present in some cases. These have a pronounced tendency to recur, but they are benign. The tumor is composed of a fibrous mass that usually occupies most or all of the dermis. Intracytoplasmic eosinophilic inclusions within fibroblasts or myofibroblasts are the diagnostic feature. Such structures may be found in an apparent extracellular location, but these may actually be within fibroblasts cut in cross-section.

Infantile digital fibromas may develop to a size of 2cm, they are safe and do not usually cause any symptoms if they rub on the neighboring toe or footwear. Pale, hard, poorly circumscribed, nontender nodule with a firm broad-base. The most common treatment is surgical excision. There is a high recurrence rate of about 60%. The tumour in extra digital locations of adults does not seem to recur after excision. Anti-inflammatory agents may induce regression of dermal infiltrative lesions. Intralesional corticosteroids or fluorouracil may prove beneficial.