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Asymptomatic Cutaneous Plaques on the Scalp and Face in an Older Adult Woman

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

A 79-year-old female patient presented with a 2-year history of asymptomatic erythematous-infiltrated patches and plaques on the right side of her scalp and face. The lesions gradually enlarged and coalesced into large plaques and nodules. Mild erosions occurred occasionally on the surface of the lesions. She denied having fever, fatigue, chills, night sweats, or weight loss.

Physical examination demonstrated ill-defined, indurated erythematous-violaceous patches, nodules, and plaques involving the surface of her head and face with sporadic erosion and brownish crusts (Figure, A). The findings of systemic reviews were unremarkable, and there was no lymphadenopathy. The complete blood cell count revealed normal white blood cell count. The biopsy from an erythematous nodule displayed sheets of medium to large mononuclear cells infiltrated in the dermis, some of which filled and expanded apparent dermal blood vessels and had large, irregular, pleomorphic nuclei (Figure, B). In addition, some neoplastic cells formed a balloon appearance. No epidermotrophism was found. The initial immunohistochemical stain revealed the tumor cells were positive for CD3 and negative for CD20, AE1/AE3, CK20, chromogranin A, and melan-A. The Ki-67 labeling index was 70%.

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A. Intravascular anaplastic large cell lymphoma

Further investigation showed that more than 90% of neoplastic cells stained positive for CD4, CD30, and LEF1 and negative for CD8, CD79a, EBER, CD56, ALK, EMA, TIA-1, and granzyme, suggestive of anaplastic large T-cell phenotype. Immunostaining for CD31 and D2-40 highlighted the vessel walls, and surprisingly, the staining pattern outlined the intravascular nature of all the neoplastic cells (Figure, C). Results of fluorescence in situ hybridization analysis performed on 4-μm tissue sections revealed 6p25.3 rearrangements (DUSP22 rearrangement). Positron emission tomography imaging did not show substantial lymphadenopathy or evidence of visceral involvement. The patient was referred to the Department of Hematology for further management, but she refused and then was lost to follow-up.

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Article Information

Corresponding Authors: Yiqun Jiang, MD (yiqunjiang@qq.com), and Hao Chen, MD (ch76ch@163.com), Department of Dermatopathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, #12 Jiangwangmiao Rd, Nanjing, Jiangsu 210042, China.

Published Online: July 21, 2022. doi:10.1001/jamaoncol.2022.2558

Conflict of Interest Disclosures: None reported.

Funding/Support: This work was supported by CAMS Innovation Fund for Medical Sciences (2021-I2M-C&T-B-087).

Role of the Funder/Sponsor: The funder had no role in the preparation, review, or approval of the manuscript and decision to submit the manuscript for publication.

Additional Contributions: We thank the patient for granting permission to publish this information. We also thank Jianfang Sun, MD, from the Department of Dermatopathology, Institute of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, for giving advice about diagnosing this case. This individual was not compensated.

References
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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.

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