Interactive Case Study - November 2024

19-year-old pregnant female with a bilateral lower extremity rash

Mavra Masood, MD

Freba Farhat, MD

Fnu Nutan, MD

An otherwise healthy 19-year-old G1P0 female at 30 weeks gestation presented with a reported painful bilateral lower extremity rash. The rash first started at 27 weeks gestation as violaceous patches on the right leg then slowly progressed to both legs and began to ulcerate. She also reported arthralgias of the bilateral hips. Physical exam reveals bilateral reticular violaceous patches on thighs with superficial ulcerations (Figure 1 and 2). Laboratory evaluation is significant for negative for anti-nuclear antibody (ANA), antiphospholipid antibody, and anti-double stranded-DNA antibody. She also had normal coagulation studies (PT, PTT) and undetectable cryoglobulins. Dermatologic evaluation consisted of two 8-millimeter punch biopsies for hematoxylin and eosin (H&E) staining as well as tissue cultures. The histopathology is demonstrated below (Figure 3 and 4). Treatment with a three-week prednisone taper, aspirin, and low molecular weighted heparin (LMWH) injections resulted in significant improvement of the lesions.

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Figure 1

Figure 2

Figure 3

Figure 4

Which of the following is the most likely diagnosis?

Cutaneous angiosarcoma

Epithelioid hemangioma

Kaposi sarcoma

Reactive angioendotheliomatosis

Spindle cell hemangioma

Which is the most appropriate next step?

Additional serologic testing

Imaging

Radiation therapy

Reassurance

Surgical excision

Which of the following histopathological findings characterize reactive angioendotheliomatosis?

Cannonball-like proliferation of blood vessels in the dermis with peripheral crescentic shaped vessels

Dilated blood vessels in the dermis and subcutaneous fat filled with large, atypical discohesive cells, hyperchromatic nuclei and numerous mitotic figures

Lobular proliferation of capillaries with prominent neutrophilic infiltrate and purplish bacterial colonies

Perivascular and periappendageal lymphocytic infiltrate in the superficial and deep dermis with abundant dermal mucin deposition

Poorly circumscribed, tightly packed capillaries without significant atypia found in the dermis with fibrin thrombi

McMenamin ME, Fletcher CD. Reactive angioendotheliomatosis: a study of 15 cases demonstrating a wide clinicopathologic spectrum. Am J Surg Pathol. 2002 Jun;26(6):685-97. doi: 10.1097/00000478-200206000-00001. PMID: 12023572.
Bin Saif GA, Buraik MA, Pokharel A, Sangueza OP, Ghada Bin Saif CA. Recurrent reactive angioendotheliomatosis in pregnancy: a case report.
Johnson NM, Shvygina A, Luyimbazi DT, Grider DJ. Rare mimic of angiosarcoma: Erythema ab igne with reactive angiomatosis. J Cutan Pathol. 2021;48(10):1298-1302. doi:10.1111/cup.14072
Thai KE, Barrett W, Kossard S. Reactive angioendotheliomatosis in the setting of antiphospholipid syndrome. Australas J Dermatol. 2003;44(2):151-155. doi:10.1046/J.1440-0960.2003.00670.X
Elman SA, González-Barreto RM, Mihm MC, Imadojemu S. Purpuric plaques in a patient with breast cancer. JAAD Case Reports. 2020;6(12):1228. doi:10.1016/J.JDCR.2020.09.013
Requena L, Sangueza OP. Cutaneous vascular proliferations. Part III. Malignant neoplasms, other cutaneous neoplasms with significant vascular component, and disorders erroneously considered as vascular neoplasms. J Am Acad Dermatol. 1998;38(2 Pt 1):143-175. doi:10.1016/S0190-9622(98)70237-3
Ferreli C, Atzori L, Rongioletti F. Glomeruloid reactive angioendotheliomatosis in a woman with systemic lupus erythematosus and antiphospholipid syndrome mimicking reticular erythematous mucinosis. JAAD Case Reports. 2021;8:56. doi:10.1016/J.JDCR.2020.12.012

Acknowledgements

We would like to acknowledge Dr. Jennifer Crimmins for her help in editing the case manuscript and assisting with the pathologic diagnosis and discussion.

Author Profiles

Mavra Masood, MD

Virginia Commonwealth University School of Medicine

Virginia Commonwealth University School of Medicine
masood.mavra@gmail.com
804-928-8143
Mavra is a first year internal medicine resident at Lankenau Medical Center. She graduated from the Virginia Commonwealth University School of Medicine. She earned her bachelor’s degree in global public health at the University of Virginia. She is currently applying to dermatology residency programs and is interested in medical education research and health disparities.

Freba Farhat, MD

Virginia Commonwealth University Health System Department of Dermatology

Dr. Freba Farhat is currently a 4th year dermatology resident at VCU Health System. She earned her medical degree at Drexel University College of Medicine. Dr. Farhat has a great interest in medical education and hopes to pursue a career in academia.

Fnu Nutan, MD

Virginia Commonwealth University Health System Department of Dermatology

Assistant Professor of Dermatology and Medicine. Dr. Nutan finished her medical education and dermatology training in India. She also received training in internal medicine from Baltimore at Medstar Good Samaritan Hospital (affiliated with
Johns Hopkins University). She currently runs the inpatient dermatology service at VCU. Her areas of interest are severe cutaneous adverse reactions (SCARs), mentoring and giving back to the community.

Question:

Interactive Case Study - November 2024

Acknowledgements

Author Profiles

19-year-old pregnant female with a bilateral lower extremity rash

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