DFSP is an uncommon mesenchymal neoplasm originating in the dermis. About 1,000 cases have been reported since DFSP was first described. It usually occurs as an indurated plaque that slowly increases in size and develops multiple firm nodules. Rarely, the initial plaque may be atrophic or depressed, and this atrophic appearance may persist. Lambert et al first described 5 cases that resembled morphea or morpheaform BCC but showed typical DFSP on biopsy. They suggested that the term protuberans should be discarded from the name dermatofibrosarcoma protuberans. Atrophic DFSP is an uncommon clinical variant of DFSP. To our knowledge, there have been 31 reported cases of atrophic DFSP to date . While atrophic DFSP has a distinct clinical appearance, its epidemiology, histology and clinical behavior appear to be similar to the common protuberant type. Truncal involvement is seen in 79% of cases, and there is a slight female predominance (62%), with the average age of onset being 30 years. It presents as a large, irregularly outlined, tan to brownish depressed scar-like lesion with atrophic patch. It is generally asymptomatic, slow growing, and often benign appearance, thus diagnosis is frequently delayed (median delay 6 years) . It may be clinically confused with other atrophic or sclerotic dermato- logic conditions, such as morphea, anetoderma, morpheaform basal cell carcinoma, scar, and lym- phocytoma.

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A 33-year-old female presented with gray de­pressed atrophic lesion accompanying a skin-colored pea-sized nodule arising from it, and erythematous indurated plaque with intermittent itching and pricking on her upper back. She had first noticed an asymptomatic erythematous plaque 7 years ago. Later, a skin-colored nodule had developed on the left lateral rim of that lesion and other part became depressed with grayish discoloration. And then an erythematous indurated plaque newly developed below the preexisting lesion (Fig. 1).

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INTRODUCTION

Dermatofibrosarcoma protuberans (DFSP) is a slow-growing, locally aggressive tumor marked by its high rate of local recurrence. It usually begins as a red-brown indurated plaque, and develops nodules slowly over many years. Rarely, the initial skin lesion may be atrophic or depressed, and may persist despite the advance stage of tumor. Atrophic DFSP is a rare variant of DFSP, which presents as a depressed violaceous scar-like lesion with atrophic patch. The biologic behavior and histologic charac­teristics of atrophic DFSP is identical to classical DFSP. Read the rest of this entry »