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Facial Erythema in an Elderly Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A man in his 80s with a pacemaker; a history of congestive heart failure, coronary artery disease, atrial fibrillation, transient ischemic attack, and Parkinson disease; and dependence in all activities of daily living presented to the dermatology department with a 4-month history of new-onset persistent facial eruption. He denied a history of facial flushing. The patient was initially treated for rosacea at an outside hospital with topical 1% metronidazole cream for 1 month without improvement and developed acute facial purpura after 1 day of treatment with oral doxycycline, which was discontinued. Because of the eruption’s rapid onset and violaceous appearance, as well as empirical treatment failure, the patient was referred for further evaluation. On examination, the patient had asymmetric, centrofacial, erythematous-violaceous indurated telangiectatic and ecchymotic plaques over a phymatous background (Figure, A). A series of punch biopsies were performed (Figure, B-D).

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C. Rosacea-like angiosarcoma

Microscopic examination findings revealed a dissecting vascular proliferation through the entire dermis. Ectatic vascular channels were lined by atypical plump endothelial cells (Figure, B and C). Immunohistochemistry staining results were negative for Human herpesvirus 8 (Figure, D). These findings were diagnostic of cutaneous angiosarcoma (CA).

Results of a positron emission tomography/computed tomography scan revealed only mildly avid malar subcutaneous thickening consistent with CA (American Joint Committee on Cancer tumor-node-metastasis staging T2aN0M0G2, stage IIB). When feasible, standard treatment entails surgery with or without radiation; however, owing to this patient’s comorbidities, poor performance status, and disease extent, reduced-dose intravenous paclitaxel (60 mg/m2) was initiated as palliative therapy. After completing 6 months of 120-mg paclitaxel weekly treatment, a partial response was obtained. Because of worsening overall health, no additional treatments were added. He was transitioned to home hospice.

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Article Information

Corresponding Author: Alina Markova, MD, Dermatology Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, 545 E 73rd St, New York, NY 10021 (markovaa@mskcc.org).

Published Online: March 25, 2020. doi:10.1001/jamadermatol.2020.0123

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient and his executor for granting permission to publish this information. From the Memorial Sloan Kettering Cancer Center, New York, NY, thanks to Arlyn Apollo, MD (Medical Oncology Service), Cristopher Barker, MD (Radiation Oncology Service), and Ciara Kelly, MD (Department of Medical Oncology), for their invaluable help with patient care and for reviewing an earlier version of the article. They were not compensated.

References
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Albores-Saavedra  J , Schwartz  AM , Henson  DE ,  et al.  Cutaneous angiosarcoma: analysis of 434 cases from the Surveillance, Epidemiology, and End Results program, 1973-2007.   Ann Diagn Pathol. 2011;15(2):93-97. doi:10.1016/j.anndiagpath.2010.07.012PubMedGoogle ScholarCrossref
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Guan  CS , Wang  XM , Lv  ZB , Yan  S , Sun  L , Xie  RM .  MRI findings of AIDS-related giant facial Kaposi’s sarcoma: a case report.   Medicine (Baltimore). 2018;97(41):e12530. doi:10.1097/MD.0000000000012530PubMedGoogle Scholar
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Hengge  UR , Ruzicka  T , Tyring  SK ,  et al.  Update on Kaposi’s sarcoma and other HHV8 associated diseases, part 1: epidemiology, environmental predispositions, clinical manifestations, and therapy.   Lancet Infect Dis. 2002;2(5):281-292. doi:10.1016/S1473-3099(02)00263-3PubMedGoogle ScholarCrossref
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Danesh  MJ , Chang  AL .  The role of the dermatologist in detecting elder abuse and neglect.   J Am Acad Dermatol. 2015;73(2):285-293. doi:10.1016/j.jaad.2015.04.006PubMedGoogle ScholarCrossref
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Hu  SC , Chen  GS , Lu  YW , Wu  CS , Lan  CC .  Cutaneous metastases from different internal malignancies: a clinical and prognostic appraisal.   J Eur Acad Dermatol Venereol. 2008;22(6):735-740. doi:10.1111/j.1468-3083.2008.02590.xPubMedGoogle ScholarCrossref
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Jia  QN , Jin  HZ , Zeng  YP .  An elderly woman with violaceous swelling with telangiectasia and necrosis on the face.   JAMA Dermatol. 2019;155(5):615-616. doi:10.1001/jamadermatol.2018.5316PubMedGoogle ScholarCrossref
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Dobson  CM , Tagor  V , Myint  AS , Memon  A .  Telangiectatic metastatic breast carcinoma in face and scalp mimicking cutaneous angiosarcoma.   J Am Acad Dermatol. 2003;48(4):635-636. doi:10.1067/mjd.2003.256PubMedGoogle ScholarCrossref
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AMA CME Accreditation Information

Credit Designation Statement: The American Medical Association designates this Journal-based CME activity activity for a maximum of 1.00  AMA PRA Category 1 Credit(s)™. Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to:

  • 1.00 Medical Knowledge MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program;;
  • 1.00 Self-Assessment points in the American Board of Otolaryngology – Head and Neck Surgery’s (ABOHNS) Continuing Certification program;
  • 1.00 MOC points in the American Board of Pediatrics’ (ABP) Maintenance of Certification (MOC) program;
  • 1.00 Lifelong Learning points in the American Board of Pathology’s (ABPath) Continuing Certification program; and
  • 1.00 CME points in the American Board of Surgery’s (ABS) Continuing Certification program

It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting MOC credit.

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