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Painful Mucosal Ulcerations in a Patient With HIV

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 40s with a long-standing history of poorly controlled HIV (multiple treatment lapses, viral load of 22 871 copies/mL 8 months prior to presentation but now undetectable with use of highly active antiretroviral therapy, with a CD4 count of 40 cells/mm3) presented to the emergency department with nausea, abdominal pain, bloody stools, odynophagia, and recurrent oral and perianal ulcers and was found to have pancytopenia and septic shock. During admission in the intensive care unit, treatment with broad-spectrum oral antibiotics for presumed infection and oral fluconazole for thrush led to improvement in the patient’s medical condition, but the ulcers persisted. Dermatology was consulted for input on the cause of the ulcers, which had been ongoing for approximately 6 months prior; individual lesions lasted 1 to 3 weeks before self-resolving. Biopsy of an oral mucosal lesion performed 1 month earlier had shown nonspecific inflammation. Examination revealed exquisitely tender, ovoid, punched-out ulcerations of the inferior lip and scrapeable white plaques on the tongue (Figure, A), right buccal mucosa, and external anal mucosa (Figure, B). A punch biopsy from 1 of the perianal ulcers was performed (Figure, C and D).

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B. Epstein-Barr virus–positive mucocutaneous ulcers

Histopathologic evaluation showed ulcerated squamous epithelium with prominent mixed infiltrate of histiocytes, neutrophils, lymphocytes, and eosinophils, with prominent admixed large and atypical cells, some of which contained larger, atypical nuclei. Gram, acid-fast bacilli, and periodic acid–Schiff diastase results were all negative for organisms, as were herpes simplex virus type 1 (HSV-1) and HSV-2 stains. An Epstein-Barr virus (EBV)–encoded RNA (EBER) in situ hybridization study was performed and demonstrated positivity in scattered lymphocytes that raised concern for EBV-positive mucocutaneous ulcers. Bone marrow biopsy was performed and did not show concerning abnormalities. The patient continued treatment with highly active antiretroviral therapy for HIV with some improvement in CD4 count to 62 cells/mm3 1 month following discharge, and the thrush resolved with oral fluconazole. The ulcers resolved without reoccurrence between discharge and follow-up 3 months later.

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Article Information

Corresponding Author: Ayan Kusari, MD, Department of Dermatology, University of California, San Francisco School of Medicine, 1701 Divisadero St, 4th Floor, San Francisco, CA 94115 (ayan.kusari@ucsf.edu).

Published Online: June 8, 2022. doi:10.1001/jamadermatol.2022.2034

Conflict of Interest Disclosures: None reported.

Disclaimer: Dr Chang is Viewpoint Editor of JAMA Dermatology but was not involved in any of the decisions regarding review of the manuscript or its acceptance.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Hadley Pearson, MD (University of California, San Francisco), for involvement in the clinical care of this patient. Dr Pearson was not compensated for her contribution.

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