eMedicine Specialties > Dermatology > Benign Neoplasms

Dermatofibroma: Differential Diagnoses & Workup

Author: Joseph C Pierson, MD, Consulting Staff, Department of Dermatology, Guthrie Army Clinic, Ft Drum, NY
Coauthor(s): Christine C Tam, MD, Staff Physician, Dermatology Office of David A Spott, MD
Contributor Information and Disclosures

Updated: Oct 2, 2009

Differential Diagnoses

Atypical Mole (Dysplastic Nevus)
Mastocytosis
Basal Cell Carcinoma
Metastatic Carcinoma of the Skin
Blue Nevi
Neurilemoma
Dermatofibrosarcoma Protuberans
Nevi, Melanocytic
Juvenile Xanthogranuloma (Nevoxanthoendothelioma)
Pilomatrixoma
Keloid and Hypertrophic Scar
Prurigo Nodularis
Keratoacanthoma
Spitz Nevus
Leiomyoma
Squamous Cell Carcinoma
Malignant Melanoma

Other Problems to Be Considered

Cutaneous chondroma
Desmoplastic trichoepithelioma
Foreign body granuloma
Granular cell tumor
Nodular fasciitis
Nodular scabies
Sclerosing sweat duct carcinoma

Workup

Procedures

  • For those trained in dermoscopy, this may be a useful adjunctive diagnostic technique for dermatofibromas (DFs). The most common pattern seen is a peripheral pigment network with a central white area.19 Dermoscopy of a xanthomatous dermatofibroma shows a homogeneous pattern with shades of yellow and a peripheral pigment network.20 If a suggestive melanocytic pattern is noted with dermoscopy, a diagnostic biopsy is warranted.
  • If any diagnostic uncertainty exists, excisional biopsy with removal of the subcutaneous fat should be performed.
  • Dermatofibroma can mimic a malignant lesion on fluorodeoxyglucose positron-emission tomography (FDG-PET) scans.21

Histologic Findings

The overlying epidermis is usually acanthotic. Pseudoepitheliomatous hyperplasia and a basaloid proliferation may be noted. The hyperplasia may be caused by the action of fibroblasts on epidermal keratinocytes.22  Basal cell carcinomas occurring upon a dermatofibroma have been reported. Increased pigment may be seen, which may be iron or melanin. Most lesions display a grenz zone of normal papillary dermis overlying the tumor.

The bulk of the tumor is within the mid dermis where no capsule is present and the periphery of the lesion blends with the surrounding tissue. Whorling fascicles of a spindle cell proliferation with excessive collagen deposition are characteristic. At the periphery, the spindle cells characteristically wrap around normal collagen bundles (see Media Files 2-3). Occasionally, melanocytes have been reported to be interspersed amongst the spindle cells.23

Acanthotic epithelium with basilar hyperpigmentat...

Acanthotic epithelium with basilar hyperpigmentation (dirty feet) over a dermal spindle cell proliferation (X10). Courtesy of David Barnette, MD.

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Acanthotic epithelium with basilar hyperpigmentat...

Acanthotic epithelium with basilar hyperpigmentation (dirty feet) over a dermal spindle cell proliferation (X10). Courtesy of David Barnette, MD.


Collagen trapping by the dermal fibrohistiocytic ...

Collagen trapping by the dermal fibrohistiocytic infiltrate (X40). Courtesy of David Barnette, MD.

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Collagen trapping by the dermal fibrohistiocytic ...

Collagen trapping by the dermal fibrohistiocytic infiltrate (X40). Courtesy of David Barnette, MD.


The subcutis typically is preserved, but if involved (especially when a storiform [cartwheel] pattern is observed), be alert to the possibility of the lesion being a dermatofibrosarcoma protuberans (DFSP). Antibodies toward factor XIIIa and CD34 may be useful in distinguishing the two tumors, with the former suggesting dermatofibroma and the latter suggesting DFSP.24 Stromelysin-3 expression of dermatofibroma by immunohistochemical staining may also be useful in distinguishing between the two.25 Indeterminate cases of tumors that share histologic and immunohistochemical features of both dermatofibroma and DFSP have been described.26 Transforming growth factor-beta type I and type II receptor expression patterns may also help distinguish between dermatofibroma and DFSP.5 Nestin, expressed in 13% of dermatofibroma and 94% of DFSP, may be another useful marker to distinguish between these 2 tumors.27

Two variants have been reported relatively frequently on the hands and feet. One shows schwannomalike features with lamellar and storiform fibrosis and a palisading of fibroblasts. The other is a myxoid variant, which appears to localize to the fingers and toes and is thought to share an association with mucous cyst of the finger.

One clinicopathologic classification scheme28 describes the following 4 categories of dermatofibroma: (1) those with architectural peculiarities, such as deep penetrating, atrophic, giant, aneurysmal (angiomatoid), hemangiopericytomalike, palisading, or ossifying variants; (2) cellular/stromal dermatofibromas, such as clear cell, granular cell, myofibroblastic, sclerotic, monster cell, atypical (pseudosarcomatous), elusive (hemosiderotic), cholesterotic (lipidized), and myxoid variants; (3) dermatofibromas with architectural and cellular/stromal changes in homogeneous arrangement, including epithelioid cell, cellular benign, smooth muscle proliferative, basal cell carcinoma–like, pseudolymphomatous, multinucleate cell angiohistiocytoma, cellular neurothekeoma, plexiform fibrohistiocytic tumor, plexiform xanthoma, and plexiform xanthomatous tumor subtypes; and (4) a complex, composite or combined dermatofibroma category with 2 or more architectural and cellular/stromal patterns in a single lesion.

Of the variants listed above, keep in mind that the uncommon sclerotic fibromalike dermatofibroma should be differentiated from sclerotic fibroma. A 2005 study29 showed 7 of 7 of the former lesions to be negative for CD34 and CD99, while 3 of 3 solitary fibromas were positive for CD34 and CD99. For comparison, 14 of 14 "common-type" dermatofibromas in this study were negative for CD34, while 4 demonstrated positivity with CD99.
In one study, CD10 was positive in 11 of 11 dermatofibromas and only positive in 1 of 7 epithelioid dermatofibromas, so it was postulated that epithelioid dermatofibroma may be a distinct entity.30

Lichenoid dermatofibroma, ulcerated dermatofibroma, erosive dermatofibroma, diffuse eosinophilic infiltrate dermatofibroma, dermatofibroma accompanied by perforating dermatosis, dermatofibroma with overlying sebaceous hyperplasia, dermatofibroma with intracytoplasmic globules,31 and signet-ring cell dermatofibroma32 have also been reported. A case of an apocrine gland cyst with a hemosiderotic dermatofibroma was termed an apocrine hemosiderotic dermatofibroma.33

A 2005 series34 reported the uncommon occurrence of dermatofibroma and melanocytic lesions in the same biopsy specimen. Four of 14 specimens showed the 2 processes to seemingly merge imperceptibly. The lesions included junctional, dermal, and compound nevi as well as a single case of melanoma in situ. Knowledge of this relationship can help prevent rendering the wrong diagnosis and is facilitated by the use of immunohistochemistry, with the melanocytic lesions showing S-100 and Mart-1 positivity with FXIIIa negativity and the dermatofibroma showing S-100 and Mart-1 negativity and FXIIIa positivity. A subsequent case report35 documenting an invasive melanoma occurring in association with a dermatofibroma underscores the role of these immunohistochemical stains.

INI-1, present in 100% of dermatofibroma and 13% of epithelioid sarcoma, can help distinguish between these 2 lesions.36

More on Dermatofibroma

Overview: Dermatofibroma
Differential Diagnoses & Workup: Dermatofibroma
Treatment & Medication: Dermatofibroma
Follow-up: Dermatofibroma
Multimedia: Dermatofibroma
References
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Further Reading

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Keywords

dermatofibroma, benign fibrous histiocytoma, dermal dendrocytoma, dermatofibroma lenticulare, fibroma durum, fibroma simplex, histiocytoma, histiocytoma cutis, nodular subepidermal fibrosis, sclerosing angioma, sclerosing hemangioma

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Contributor Information and Disclosures

Author

Joseph C Pierson, MD, Consulting Staff, Department of Dermatology, Guthrie Army Clinic, Ft Drum, NY
Joseph C Pierson, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Christine C Tam, MD, Staff Physician, Dermatology Office of David A Spott, MD
Christine C Tam, MD is a member of the following medical societies: American Medical Association, Phi Beta Kappa, and Phi Kappa Phi
Disclosure: Nothing to disclose.

Medical Editor

Terry L Barrett, MD, Clinical Professor of Dermatology and Pathology, University of Texas Southwestern School of Medicine; Director, ProPath Dermatopathology, Dallas, Texas
Terry L Barrett, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, College of American Pathologists, and United States and Canadian Academy of Pathology
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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