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Case Reports
. 2023 Jun;8(2):NP1-NP3.
doi: 10.1177/23971983231152342. Epub 2023 Feb 5.

A purple plaque in a patient with systemic sclerosis

Sheena Ramyead  1 , Christopher P Denton  2 , Catherine H Orteu  1 , Victoria Swale  3 , Jorge Mayor-Jerez  3 , Emma Gardette  1
Affiliations
Case Reports

A purple plaque in a patient with systemic sclerosis

Sheena Ramyead et al. J Scleroderma Relat Disord. 2023 Jun.

Abstract

We present the case of a 43-year old woman with anti-U3 ribonucleoprotein antibody-positive systemic sclerosis presenting with an enlarging purple plaque on the left upper arm. The skin was not sclerotic; however, there had been a cluster of long-standing telangiectases preceding the plaque. Histology and immunohistochemistry confirmed an angiosarcoma. There are five reported cases in the literature about angiosarcoma arising in the skin of patients with systemic sclerosis; however, to our knowledge, this is the first to have arisen from non-sclerotic skin. We would urge clinicians to adopt a high index of suspicion for atypical vascular tumours presenting in patients with systemic sclerosis.

Keywords: Systemic sclerosis; angiosarcoma; connective tissue disease; skin cancer; vascular tumour.

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Conflict of interest statement

The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.

Figures

Figure 1.
Figure 1.
(a) A large purple plaque on the left upper arm with surrounding telangiectasia. (b) A closer view of the plaque with a central nodule.
Figure 2.
Figure 2.
(a) The tumour consists of vascular channels lined by atypical endothelial cells, with nuclear pleomorphism and increased mitotic activity. (b) The atypical endothelial cells show strong diffuse expression with CD31 (immunoperoxidase).
See this image and copyright information in PMC

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