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Hematology

Widespread Erythematous Plaques With Prominent Telangiectases

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

1 Credit CME

JAMA Dermatology

Published Online: October 13, 2021

JAMA Dermatology Clinicopathological Challenge

Article Information and Disclosures

Ayan Kusari, MD¹;

Philip E. LeBoit, MD^1,2,3;

Anna Haemel, MD¹

Author Affiliations

¹Department of Dermatology, University of California, San Francisco School of Medicine
²Department of Pathology, University of California, San Francisco School of Medicine
³Helen Diller Family Cancer Center, University of California, San Francisco School of Medicine

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A woman in her 50s with a history of nasal-type natural killer (NK)/T-cell lymphoma (diagnosed 2 years prior; treated with pembrolizumab and methotrexate, ifosfamide, etoposide, dexamethasone, and pegaspargase) and chronic pulmonary coccidiomycosis being treated with fluconazole was admitted for fever, hypotension, and rash. Three months prior, pembrolizumab treatment was stopped because of a widespread morbilliform reaction, which had resolved completely 6 to 8 weeks before admission. The new widespread, asymptomatic skin eruption had been ongoing for 1 to 2 weeks and appeared different from her pembrolizumab reaction.

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B. Intravascular involvement by NK/T-cell lymphoma

In addition to large CD3⁺/CD56⁺ perivascular lymphocytes in the dermis, there were vessels in the subcutaneous fat with intravascular lymphocytes, with a CD3⁺/CD56⁺ immunophenotype. A diagnosis of intravascular NK/T-cell lymphoma, nasal type, was made; an Epstein-Barr virus–encoded RNA in situ hybridization study had been performed on a prior biopsy, and the result was positive. Results of CD30 immunostaining were negative. Patients with lymphoma may experience fevers owing to common or atypical infections, drugs, or as a direct consequence of lymphoma. Accordingly, a careful drug review and an extensive infectious workup were undertaken. Special stains (Fite, PAS-D, Brown-Brenn) and tissue cultures were performed and results were negative, as the clinical presentation was of fever of unknown origin.

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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: October 13, 2021. doi:10.1001/jamadermatol.2021.4068

Conflict of Interest Disclosures: Dr Haemel reported personal fees from CSL Behring for serving on a data and safety monitoring board and from Guidepoint Global for serving as a consultant outside the submitted work. No other disclosures were reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Ritesh Agnihothri, MD, for providing the clinical image.

References

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Namekawa M , Nakano I . Diagnosis of intravascular lymphoma. Article in Japanese. Brain Nerve. 2011;63(5):451-458.PubMed Google Scholar

Matsue K , Abe Y , Kitadate A , et al. Sensitivity and specificity of incisional random skin biopsy for diagnosis of intravascular large B-cell lymphoma. Blood. 2019;133(11):1257-1259.PubMed Google Scholar Crossref

Hope CB , Pincus LB . Primary cutaneous B-cell lymphomas with large cell predominance–primary cutaneous follicle center lymphoma, diffuse large B-cell lymphoma, leg type and intravascular large B-cell lymphoma. Semin Diagn Pathol. 2017;34(1):85-98.PubMed Google Scholar Crossref

Adachi Y , Kosami K , Mizuta N , et al. Benefits of skin biopsy of senile hemangioma in intravascular large B-cell lymphoma. Oncol Lett. 2014;7(6):2003-2006.PubMed Google Scholar Crossref

Kwong Y-L , Kim WS , Lim ST , et al. SMILE for natural killer/T-cell lymphoma. Blood. 2012;120(15):2973-2980.PubMed Google Scholar Crossref

Wang L , Li L-R , Zhang L , Wang J-W . The landscape of new drugs in extranodal NK/T-cell lymphoma. Cancer Treat Rev. 2020;89:102065.PubMed Google Scholar

Wei W , Wu P , Li L , Zhang ZH . Effectiveness of pegaspargase, gemcitabine, and oxaliplatin (P-GEMOX) chemotherapy combined with radiotherapy in newly diagnosed, stage IE to IIE, nasal-type, extranodal natural killer/T-cell lymphoma. Hematology. 2017;22(6):320-329.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 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.