CLINICAL HISTORY:

Signs and symptoms:  Lesion was slightly tender to palpation

Previous Treatment:  None

Other information:  The patient had a past medical history significant for Milroy’s disease (Congenital Lymphedema). She reported that the lesion began as a brown patch and slowly enlarged to a dome-shaped nodule over several years. She also reported multiple similar, but smaller lesions on her upper and lower extremities and trunk. The patient was only taking Hydrochlorothiazide for the lower extremity edema.

PHYSICAL EXAM:

A 3.0 X 3.2 cm, slightly hyperpigmented, soft, pedunculated nodule was present on the left dorsal foot. A total of nine hyperpigmented, firm 0.5 – 1.0 cm nodules were found on bilateral upper and lower extremities and trunk.

LABORATORY TESTS:

A shave biopsy of the left dorsal foot lesion was done.

DERMATOHISTOPATHOLOGY:

Microscopic description: Spindle cell tumor composed of fibroblasts, histiocytes, and multinucleated giant cells in the dermis. The overlying epidermis is hyperplastic with elongation of rete ridges.

DIFFERENTIAL DIAGNOSIS:

1.   Polypoid nodular dermatofibroma
2.   Superficial leiomyoma
3.   Keloid
4.   Malignancy
5.   Acquired Fibrokeratoma

SCROLL DOWN FOR ANSWER AND DISCUSSION.

CORRECT DIAGNOSIS:

Polypoid nodular dermatofibroma

DISCUSSION:

Dermatofibroma, also known as benign fibrous histiocytoma, is a common benign cutaneous neoplasm. Typically dermatofibromas present as firm, dome shaped nodules and usually are only a few millimeters in diameter. Atypical polypoid nodular dermatofibroma is a rare variant of dermatofibroma first described in 1991. Polypoid dermatofibromas most often arise on the lower extremities and are larger than typical dermatofibromas. It is postulated that firm underlying tissue such as tendons and bones leads to the development of exophytic polypoid lesions.

Primary lymphedema is divided into three groups based on age of presentation. Congenital lymphedema is an autosomal dominant condition present at birth and is known as Milroy’s disease. We speculate that compromised lymphatic flow contributed to the pathogenesis of the polypoid dermatofibroma. This is the first reported case of Milroy’s disease associated with polypoid dermatofibroma.

TREATMENT:

Actual treatment for this patient: The lesion was surgically excised with no recurrence at 3-month follow up

Other Treatment options: Dermatofibromas may be treated with excision, potent topical steroids, and intralesional steroids.

REFERENCES:

1. Sogabe et al. A Case of Polypoid Dermatofibroma.Journal of Dermatology 2002 Dec;12:786-9.

2. Puig et al. Atypical polypoid dermatofibroma:Report of two cases.JAAD 1991 Apr; 4:561-5.

3. Sehgal et al. Giant combined dermatofibroma with satellitosis. Clin Exp Dermatol.2004 Mar; 29(2):147-9.

4. Setoyama et al. Case of dermatofibroma with monster cells: a review and immunohistochemical study. Am J Dermatopath. 1997 Jun;19(3):312-5.

5. Lever WF et al. Histopathology of the Skin, 7th ed. Philadelphia, JB Lippincott, 1990.

Additional Comment:

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