Table of Contents
Definition and Epidemiology of Dermatofibromas
Dermatofibroma is a common mesenchymal growth of the skin where skin fibroblasts are the major cell constituents. It is also known as cutaneous benign fibrous histiocytoma or sclerosing hemangioma.
A common mesenchymal tumor of the skin, dermatofibroma is known to have a similar occurrence across all races. There is a predilection for women, mostly occuring in the lower legs, and in early adulthood, from 20 to 30 years of age. It can, however, occur in any age. The estimated female to male ratio is 2.6 to 1. The average age at time of presentation is 36.2 years.
Pathophysiology of Dermatofibromas
The exact mechanism for the development of a dermatofibroma is unknown, and clinicians are divided on whether it is a reactive process or a neoplastic one. Association with trauma and insect bites have led some to believe a reparative or reactive process may be the underlying pathology. However, recent demonstrations on chromosomal abnormalities and clonal proliferation have hinted more towards a neoplastic mechanism.
More likely, however, it is said to arise from a distortion of protein kinase C activity. Immunohistochemical testing results have been found to be more consistent with the presence of factor XIIIa and fascin (dendritic cell markers) than with MAC – 387, a histocyte marker.
Mast cells are found in abnormally high numbers. They release fibrogenic factors like transforming growth factor beta (TGF beta) and trigger the fibrosis seen in dermatofibroma. In the epitheloid and atypical dermatofibroma variants specifically, ALK gene rearrangement and over-expression have been implicated.
Clinical Features of Dermatofibromas
- Measuring about 0.5-1cm in size, dermatofibromas are characteristically firm, indurated and mobile.
- The lesions may be solitary or multiple (defined as at least 15 but most commonly benign finding), generalized or neoplastic. Multiple lesions are usually associated with an immunosuppressive condition like HIV, myasthenia gravis, systemic lupus erythematosus, diabetes, etc.
- When neoplastic, they are usually confined to the soles and palms and may grow rapidly within weeks. However, some have been found to remain static for years.
- Lateral compression produces a dimple-like depression in the overlying skin which disappears with release of pressure. This is called button-holing but is not specific to dermatofibroma. Positive button hole sign is also seen in type-1 neurofibromatosis (Von-Recklinghausen’s disease) and anetoderma.
- Regression of these lesions may leave hypopigmented spots.
- Dermatofibromas arising on the face are quite uncommon but distinct from lesions seen at other sites. More commonly of the cellular type, they may extend into subcutaneous fat or muscle and recur frequently. This is why a wider initial excision is warranted in this case.
- Local recurrence is a feature of the atypical variant as well, along with distant metastasis that is observed here.
- Up to 58% of the tumors are asymptomatic. 20.5% are tender to palpation and 11.5% are painful. The most common symptom, however, is pruritic sensation or itching.
The deep variant of dermatofibroma may be confused with an entity called dermatofibrosarcoma protuberans that is rather typically found on the trunk in younger adults. The size of this lesion is much larger – about 1-2cm.
In ambiguous cases, where the histological picture of the cellular variant closely resembles that of dermatofibrosarcoma protuberans, immunolabelling can be useful in distinguishing the factor XIIIa- and fascin-positive dermatofibroma from the latter (found to be negative for these markers).
However, the following skin pathologies are also to be considered in the differential:
- Atypical fibroxanthoma
- Basal cell carcinoma
- Juvenile xanthogranuloma
- Prurigo nodularis scar
- Squamous cell carcinoma
Investigations for Dermatofibroma
While the diagnosis of dermatofibroma is usually made clinically, confirmation can be undertaken by histological analysis on biopsy.
Histological picture obtained on punch biopsy of the lesion reveals information on not just the type of variant but also helps in making crucial decisions regarding management.
A typical picture that is most often encountered is:
- Epidermis: usually hyperplastic and hyperpigmented, known as the dirty fingernail sign.
- Dermis: haphazardly arranged fascicles of plump, spindle-shaped cells that typically lack atypia.
- Edges of lesion: not well defined, with individual cells infiltrating between thickened and hylanized collagen bundles (typical keloidal picture); also known as “collagen trapping.”
- Mitotic figures may be present, but atypia is characteristically not seen.
A cellular variant shows a storiform arrangement embedded in the deep dermis, with a much larger size and increased cellularity. It may often extend into the superficial subcutaneous tissue making it difficult to distinguish from an entity called dermatofibrosarcoma protuberans.
A rare form of atypical variant shows atypical features against a background of the classic picture of dermatofibroma. Pleomorphic, spindle-shaped, plump or polyhedral-shaped cells are seen with keloidal collagen and storiform arrangement. The histopathological classification of dermatofibromas is given below:
- Fibrocollagenous (40.1%)
- Histiocytic (13.1%)
- Cellular (11.5%)
- Aneurysmal (7.4%)
- Angiomatous (6.5%)
- Sclerotic (6.5%)
- Other (14.9%)
Visualizing under a dermatoscope usually presents a peripheral pigment network with a central white area.
Indicated in cases where immunosuppressive conditions are suspected.
Management of Dermatofibroma
An intervention is usually not indicated in asymptomatic cases that constitute the majority. Watchful waiting and reassurance suffice.
For symptomatic cases:
- Medical management: This is for patients who do not desire to undergo a surgical procedure. Topical or intralesional corticosteroids may help relieve the symptoms.
- Cryosurgery is another option to flatten out the dermatofibroma but is usually not curative.
- Surgical management includes cutaneous shaving, excisional surgery and ablative laser surgery. This is usually indicated for cases where repeated trauma is suffered due to shaving on the lower legs. 20% of the cellular variant are known to recur after a simple biopsy and thus warrant a fusiform excision into the superficial subcutaneous tissue. For deeper variants, excision to the deeper subcutaneous tissue is done to decrease the risk for recurrence.
Lesions that continue to increase in size, become symptomatic or bleed, change size or symmetry are to be biopsied before establishing a management protocol.