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Excerpt from Acquired Digital Fibrokeratoma


Synonyms, Key Words, and Related Terms: ADFK, acral fibrokeratoma, acquired periungual fibrokeratoma, Koenen tumor, Koenen's tumor, garlic clove fibroma, subungual fibrokeratoma, periungual fibrokeratoma

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Background

In 1968, Bart et al1 described 10 cases of an uncommon acquired growth that was located on the fingers. Although it clinically resembled a cutaneous horn or rudimentary supernumerary digit, it had distinct histopathological findings. The authors named this growth acquired digital fibrokeratoma (ADFK). Subsequently, Pinkus2 reported 28 more cases; however, because the lesions Pinkus described also occurred on the proximal hand, toes, soles, and one in the prepatellar region, he suggested the entity might be more appropriately called acquired acral fibrokeratoma.

An acquired periungual fibrokeratoma is similar to an acquired acral fibrokeratoma, differing primarily in that the former arises from the proximal nail fold. Koenen tumors, although similar, occur in association with tuberous sclerosis and histologically may have atypical stellate myofibroblasts.3

Pathophysiology

Despite the fact that most patients deny a history of precedent trauma, the major hypothesis is that subclinical injury contributes to the development of these lesions.

Frequency

United States

Currently, no means of tracking nonmelanoma skin cancer, much less various benign dermatological conditions, are available in the United States; therefore, the actual incidence of acquired acral fibrokeratoma is unknown. Most cases reported in the literature involve individual case reports presented because of the lesions' unusual size, location, histological features, or association with other conditions. Only a few reports describe a series of patients, with 50 patients being the most reported from any one institution.4 Therefore, whether this condition is rare or rarely reported remains unclear.

Mortality/Morbidity

ADFKs are benign stationary lesions that are more cosmetically bothersome than they are problematic. However, patients who have been reported to have giant acral digital fibromas on the dorsum or plantar surface5, 6 of the foot may report some discomfort.

Race

ADFKs have been reported in persons of all races.

Sex

ADFKs seem to have a slight male predominance; however, at this time too few cases have been described to adequately assess the significance of any sexual predilection.7

Age

The patients reported with ADFKs range in age from 12-70 years, with most cases occurring in middle-aged adults. Clinically similar lesions that occur in young children are more likely to represent rudimentary supernumerary digits.

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