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Rheumatology

Erythematous Papules and Hemorrhagic Vesicles in a Woman With Arthralgias and Eye Pain

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

1 Credit CME

JAMA

Published Online: November 10, 2021

JAMA Clinical Challenge

Article Information and Disclosures

Lindsey West, MD¹;

Sung Kyung Cho, BA¹;

Cristina Thomas, MD^1,2

Author Affiliations

¹Department of Dermatology, University of Texas Southwestern Medical Center, Dallas
²Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas

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A 48-year-old woman with diet-controlled diabetes presented with a 6-month history of arthralgias in her hands, feet, elbows, and knees; 3 weeks of a purpuric rash and foot paresthesias; and 2 weeks of bilateral eye pain and redness. She had been prescribed 4 days of prednisone (10 mg/d) without improvement. She had no fevers, epistaxis, rhinorrhea, cough, shortness of breath, or hematuria. She had no history of recent travel or illicit drug use and was not taking any medications.

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ANCA-associated vasculitis

A. Check antineutrophil cytoplasmic antibody (ANCA) titers

The key to the correct diagnosis is the history of arthralgias, paresthesias, ocular inflammation, palpable purpura, and skin biopsy findings of leukocytoclasia, which is characteristic of small vessel vasculitis. ANCA-associated vasculitis due to granulomatosis with polyangiitis was confirmed with serum indirect immunofluorescence, diffuse cytoplasmic staining ANCA (c-ANCA) (+1:20), and a positive proteinase 3 (PR3) antibody (+51.8).

Chikungunya (choice B) is a mosquito-borne viral illness that may cause arthralgias and conjunctivitis; however, its rash is morbilliform rather than purpuric. Obtaining a transthoracic echocardiogram (choice C) is incorrect because without fevers or history of intravenous drug use, endocarditis is unlikely. While Chlamydia trachomatis (choice D) may cause a reactive arthritis with arthralgias and conjunctivitis, it does not cause cutaneous small vessel vasculitis.

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Article Information

Corresponding Author: Cristina Thomas, MD, Department of Dermatology, UT Southwestern Medical Center, 5323 Harry Hines Blvd, Dallas, TX 75390-9069 (cristina.thomas2@utsouthwestern.edu).

Published Online: November 10, 2021. doi:10.1001/jama.2021.18708

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for providing permission to share her information.

References

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Geetha D , Jefferson JA . ANCA-associated vasculitis: core curriculum 2020. Am J Kidney Dis. 2020;75(1):124-137. doi:10.1053/j.ajkd.2019.04.031 PubMed Google Scholar Crossref

Micheletti RG , Chiesa Fuxench Z , Craven A , Watts RA , Luqmani RA , Merkel PA ; DCVAS Investigators. Cutaneous manifestations of antineutrophil cytoplasmic antibody–associated vasculitis. Arthritis Rheumatol. 2020;72(10):1741-1747. doi:10.1002/art.41310 PubMed Google Scholar Crossref

Samman KN , Ross C , Pagnoux C , Makhzoum JP . Update in the management of ANCA-associated vasculitis: recent developments and future perspectives. Int J Rheumatol. 2021;2021:5534851. doi:10.1155/2021/5534851 PubMed Google Scholar

Geetha D , Jin Q , Scott J , et al. Comparisons of guidelines and recommendations on managing antineutrophil cytoplasmic antibody–associated vasculitis. Kidney Int Rep. 2018;3(5):1039-1049. doi:10.1016/j.ekir.2018.05.007 PubMed Google Scholar Crossref

Mendel A , Ennis D , Go E , et al. CanVasc Consensus recommendations for the management of antineutrophil cytoplasm antibody–associated vasculitis: 2020 Update. J Rheumatol. 2021;48(4):555-566. doi:10.3899/jrheum.200721 PubMed Google Scholar Crossref

Shi L . Anti-neutrophil cytoplasmic antibody–associated vasculitis: prevalence, treatment, and outcomes. Rheumatol Int. 2017;37(11):1779-1788. doi:10.1007/s00296-017-3818-y PubMed Google Scholar Crossref

Yates M , Watts R . ANCA-associated vasculitis. Clin Med (Lond). 2017;17(1):60-64. doi:10.7861/clinmedicine.17-1-60 PubMed Google Scholar Crossref

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 credit toward the CME [and Self-Assessment requirements] of the American Board of Surgery’s Continuous Certification program

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