Brittany Carter1*, Christine Jaworsky2 and Monte Fox3
1Western Reserve Hospital, Ohio University Heritage College of Osteopathic Medicine, Detroit, USA
2Department of Dermatology, Case Western Reserve University School of Medicine, University of Pennsylvania, Cleveland, USA
3Western Reserve Hospital, Summa Akron City, Cleveland Clinic Akron General, Case Western Reserve School of Medicine, Cuyahoga Falls, USA
Received: 11 August, 2016; Accepted: 26 August, 2016; Published: 27 August, 2016
Brittany Carter, DO, Resident, Western Reserve Hospital, Ohio University, Heritage College of Osteopathic Medicine, 3670 Woodward Avenue, #414 Detroit, MI 4820, Tel: 313-585-6927; E-mail:
Carter B, Jaworsky C, Fox M (2016) Systemic Sclerosis Associated Angiosarcoma: A Case Report and Review of the Literature. Int J Dermatol Clin Res 2(1): 014-017. DOI: 10.17352/2455-8605.000015
© 2016 Carter B, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Cutaneous angiosarcoma; Epithelioid angiosarcoma; Systemic sclerosis
PET: Positron Emission Tomography
Cutaneous angiosarcoma is an uncommon malignant vascular tumor with a highly variable presentation. It most commonly occurs on the head and neck of elderly Caucasian men, while less commonly on the skin of persons with pre-existing conditions or cutaneous lesions. To date, only three reports of angiosarcoma arising on the head and neck of individuals with systemic sclerosis exist in the literature. We report the fourth case: a 58 year-old African-American female with systemic sclerosis who developed an epithelioid subtype of cutaneous angiosarcoma on the right elbow, rather than the head and neck region.
Cutaneous angiosarcoma is an uncommon malignant vascular tumor with a highly variable presentation. It has a predilection for elderly Caucasian men, with 50% of tumors occurring primarily on the head and neck . Cutaneous angiosarcomas may also occur in sites with chronic lymphedema, areas of prior radiation therapy, and in association with vascular and peripheral nerve sheath tumors . Review of the literature reveals three reports of angiosarcoma arising on the head and neck of those with concomitant systemic sclerosis. There is also one report of angiosarcoma and morphea arising in a site of irradiation. We report a fourth case of angiosarcoma occurring in a patient with coexistent systemic sclerosis. Our patient has an epithelioid variant of angiosarcoma, which presented on the right elbow as an innocuous-appearing cutaneous lesion. We discuss therapeutic options and prognosis in patients with both diseases.
A 58 year-old African-American woman was referred to dermatology from rheumatology in January of 2016 for evaluation of a “wart-like lesion with skin breakdown” on the right elbow. Her past medical history was significant for progressive systemic sclerosis, end stage renal disease on hemodialysis, essential hypertension, fibromyalgia, osteochondropathy, and adhesive capsulitis of the shoulder. Surgical history was noncontributory. Her social history was negative for use of tobacco, alcohol, or recreational drugs.
Review of the patient’s records shows that in January 2015 rheumatology documented drainage from the right elbow. Cultures were positive for methicillin-sensitive staphylococcus aureus. She had started on twice-daily trimethoprim-sulfamethoxazole and warm soaks empirically, and continued for 10 days. Improvement was noted in February 2015, only to worsen soon thereafter. Concerns for possible non-infectious etiologies such as calciphylaxis and/or calcium deposits were raised. Conservative treatment continued through December 2015. At that time, further irritation, cutaneous breakdown and a new verrucous-appearing area were noted.
On presentation in dermatology, the patient’s main complaint was dry skin. Physical examination revealed an asymptomatic friable, verrucous plaque with central ulceration located on the right elbow (Figure 1). The remainder of her physical examination was notable for diffusely taut, shiny skin of the face (Figure 2), neck, trunk, upper, and lower extremities. There was superimposed generalized xerosis. Firm yellowish nodules were seen overlaying the fingers, wrists, and elbows, suggestive of calcinosis cutis. Severe contractures involving finger, wrist, elbow, and shoulder joints showed a reduced range of motion.