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Multiple Cutaneous Nodules and Limb Swelling

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 30s presented with painless swelling of the left upper limb and nodules on the left side of the chest and abdomen. The lesions had started a month previously as red nodules on the left axilla, ipsilateral chest, and back. Twenty days later, he developed edema in the left upper limb. While being evaluated for the cutaneous lesions, the patient was diagnosed with AIDS and began antiretroviral therapy.

On examination, 6 erythematous, firm, subcutaneous nodules were present on the left side of the chest, abdomen, and left upper limb, varying in size from 2 × 2 cm to 4 × 4 cm (Figure, A). There was diffuse erythema and edema on the left upper limb, leading to difficulty in limb movement (Figure, A). Multiple dilated superficial vessels were present on the posterior aspect of the left arm and ipsilateral chest wall. Left axillary lymph nodes were grossly enlarged, firm, nontender, and nonmatted, with the largest lymph node measuring 6 × 4 cm. The patient’s CD4 cell count was 141/μL (to convert to ×109/L, multiply by 0.001), and his HIV load was 504 473 copies/mL. A skin biopsy sample was obtained from the plaque and sent for histopathologic examination (Figure, B and C).

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C. Burkitt lymphoma

Skin biopsy findings revealed large atypical lymphocytes with coarse chromatin, prominent nucleoli, and a scant amount of deeply basophilic cytoplasm in the lower dermis and subcutaneous tissue. Brisk mitosis and many atypical mitotic figures were observed. The classic starry-sky pattern, an effect of benign histiocytes engulfing apoptotic tumor cells, could be seen focally in the dermis. On immunohistochemical analysis, these atypical lymphoid cells were strongly positive for CD10 and CD20 and showed nuclear positivity for c-Myc (>40%) (Figure, C), with a Ki-67 index of more than 95%. These cells were negative for CD3, CD30, terminal deoxynucleotidyl transferase (TdT), and Bcl2. The results of immunohistochemical detection of latent membrane protein 2 expression and Epstein-Barr virus (EBV) encoded RNA in situ hybridization procedures were positive. Cytogenetic analysis revealed translocation (8;14), consistent with Burkitt lymphoma (BL).

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Article Information

Corresponding Author: Dipankar De, MD, Department of Dermatology Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India (dr_dipankar_de@yahoo.in).

Published Online: February 21, 2019. doi:10.1001/jamaoncol.2018.6982

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information. We acknowledge the contribution of Divya Aggarwal, MD, Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India, for providing histopathologic images. He was not compensated for this work.

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