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Author: Elizabeth Kline Satter, MD, MPH, Staff Dermatologist and Head of Dermatopathology for Residency Program, Department of Dermatology, Naval Medical Center, San Diego

Elizabeth Kline Satter is a member of the following medical societies: Alpha Omega Alpha and American Medical Women's Association

Editors: Abby S Van Voorhees, MD, Assistant Professor, Director of Psoriasis Services and Phototherapy Units, Department of Dermatology, University of Pennsylvania School of Medicine, Hospital of the University of Pennsylvania; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Christen M Mowad, MD, Associate Professor, Department of Dermatology, Geisinger Medical Center; Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: ADFK, acral fibrokeratoma, acquired periungual fibrokeratoma, Koenen tumor, Koenen's tumor, garlic clove fibroma, subungual fibrokeratoma, periungual fibrokeratoma

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.



History

Most patients present with an asymptomatic protuberance.

Physical

Clinically, ADFKs manifest as solitary, skin-colored, dome-shaped papules or tall fingerlike protrusions with a hyperkeratotic surface. Most lesions are small and do not exceed 1.5 cm in height or diameter, but giant lesions measuring in excess of 3 cm have been documented.8

An important clinical finding reported to help differentiate ADFKs from other similar lesions is a collarette of slightly raised skin that encircles the base of the lesion, thereby creating a moatlike configuration.9

Causes

The etiology is unknown. Although trauma has been implicated, no studies can substantiate this hypothesis.

One report describes familial occurrence of an acral fibrokeratoma variant that had mucinous deposition; however, the case reported by Moulin et al10 histologically more closely resembles a superficial acral fibromyxoma.



Corns
Cutaneous Horn
Infantile Digital Fibromatosis
Pyogenic Granuloma (Lobular Capillary Hemangioma)
Supernumerary Digit
Warts, Nongenital

Other Problems to be Considered

Periungual fibromas (Koenen tumors)4
Fibroma
Superficial acral fibromyxoma
Squamous Cell Carcinoma11
Aggressive digital papillary adenocarcinoma12
 



Histologic Findings

The overall architecture of an ADFK is a small, well-circumscribed, dome-shaped or narrow elongated papule. The stratum corneum is typically hyperkeratotic, which tends to be most pronounced toward the summit of the lesion. The epidermis can be acanthotic, with elongation of the rete ridges or can be slightly attenuated.

The dermal core of an ADFK displays 1 of 3 histological patterns, which were originally described by Bart et al1 and further characterized by Kint et al.13 Type I ADFK is the most common type and consists of a dermal core composed of thick, closely intertwined collagen bundles that are often oriented along the vertical axis of the lesion. Between the collagen bundles are numerous capillaries, varying numbers of fibroblasts, and thin elastic fibers. Type II lesions are less common, and histologically resemble type I lesions, but in addition have a significantly increased number of fibroblasts arranged in fascicles and markedly  reduced numbers of elastic fibers. Type III ADFK is the least common type and consists of a dermal core that is poorly cellular and edematous with a reduced number of elastic fibers.



Surgical Care

Simple excision is curative; recurrence is rare.



Medical/Legal Pitfalls

Patients who have multiple tumors, especially on several digits, need to be evaluated for the other stigmata that characterize tuberosis sclerosis.14



Media file 1:  Clinical picture of a pedunculated acquire digital fibrokeratoma.
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Media type:  Photo

Media file 2:  Flat-topped, skin-colored acquired digital fibrokeratoma in an acral location.
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Media type:  Photo

Media file 3:  Slightly raised skin encircling the base of an acquired digital fibrokeratoma, creating a moat.
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Media type:  Photo

Media file 4:  Domed-shaped papule with overlying hyperkeratosis. The dermal core is composed of increased collagen bundles and blood vessels oriented along the vertical axis of the lesion.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo

Media file 5:  Close up showing the increased collagen bundles and blood vessels oriented along the vertical axis of the lesion.
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Media type:  Photo

Media file 6:  A different acquired digital fibrokeratoma showing similar findings of a domed-shaped lesion with a vertically aligned fibrovascular core.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Acquired Digital Fibrokeratoma excerpt

Article Last Updated: Mar 26, 2008