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Fever, Pancytopenia, and Tender Erythematous Plaques in a Patient With Multiple Myeloma

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 late 60s was referred to the dermatologic clinic with a 10-day history of fever up to 38.5 °C and tender lesions on the neck and trunk. In the previous month, he had received the first cycle of a chemotherapeutic regimen of ixazomib, lenalidomide, and dexamethasone to treat a recurrence of IgG-κ multiple myeloma with a 10-year history. Physical examination showed annular erythematous plaques of up to 2 cm in diameter, with violaceous and crusted centers on the neck and trunk. On the right chest, an annular erythematous lesion with 2 zones of color—a central area of purpura and an outer erythematous and edematous ring—was present (Figure, A). Laboratory investigations found pancytopenia: a white blood cell count of 2.3 × 103/μL (reference range, 3.5-9.8 × 103/μL), hemoglobin levels of 12.1 g/dL (reference range, 13.5-17.6 g/dL), platelet count of 29 × 103/μL (reference range, 130-400 × 103μL), and elevated serum C-reactive protein levels of 3.31 mg/dL (reference range, <0.30 mg/dL). Blood culture results were negative. Granulocyte colony-stimulating factor analogs had not been prescribed to the patient. Skin biopsy from a lesion on the left chest was performed for histopathological and immunohistochemical analyses (Figure, B-D).

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A man in his late 60s was referred to the dermatologic clinic with a 10-day history of fever up to 38.5 °C and tender lesions on the neck and trunk. In the previous month, he had received the first cycle of a chemotherapeutic regimen of ixazomib, lenalidomide, and dexamethasone to treat a recurrence of IgG-κ multiple myeloma with a 10-year history. Physical examination showed annular erythematous plaques of up to 2 cm in diameter, with violaceous and crusted centers on the neck and trunk. On the right chest, an annular erythematous lesion with 2 zones of color—a central area of purpura and an outer erythematous and edematous ring—was present (Figure, A). Laboratory investigations found pancytopenia: a white blood cell count of 2.3 × 103/μL (reference range, 3.5-9.8 × 103/μL), hemoglobin levels of 12.1 g/dL (reference range, 13.5-17.6 g/dL), platelet count of 29 × 103/μL (reference range, 130-400 × 103μL), and elevated serum C-reactive protein levels of 3.31 mg/dL (reference range, <0.30 mg/dL). Blood culture results were negative. Granulocyte colony-stimulating factor analogs had not been prescribed to the patient. Skin biopsy from a lesion on the left chest was performed for histopathological and immunohistochemical analyses (Figure, B-D).

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Article Information

Corresponding Author: Mitsuhito Ota, MD, PhD, Department of Dermatology, Chitose City Hospital, Hokkou 2, Chitose 066-8550, Japan (ota@med.hokudai.ac.jp).

Published Online: June 7, 2023. doi:10.1001/jamadermatol.2023.1450

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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Requena  L , Kutzner  H , Palmedo  G ,  et al.  Histiocytoid Sweet syndrome: a dermal infiltration of immature neutrophilic granulocytes.   Arch Dermatol. 2005;141(7):834-842. doi:10.1001/archderm.141.7.834PubMedGoogle ScholarCrossref
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Haber  R , Feghali  J , El Gemayel  M .  Risk of malignancy in histiocytoid Sweet syndrome: A systematic review and reappraisal.   J Am Acad Dermatol. 2020;83(2):661-663. doi:10.1016/j.jaad.2020.02.048PubMedGoogle ScholarCrossref
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Shabeeb RAI, Fong A, Khera P, Maiberger M. A Case of ixazomib-induced histiocytoid Sweet syndrome in a patient with multiple myeloma in a patient with multiple myeloma. March 2021. Accessed April 28, 2023. https://www.hmpgloballearningnetwork.com/site/thederm/article/case-ixazomib-induced-histiocytoid-sweet-syndrome-patient-multiple-myeloma
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Hoverson  AR , Davis  MD , Weenig  RH , Wolanskyj  AP .  Neutrophilic dermatosis (Sweet syndrome) of the hands associated with lenalidomide.   Arch Dermatol. 2006;142(8):1070-1071. doi:10.1001/archderm.142.8.1070-bPubMedGoogle ScholarCrossref
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Choong  DJ , Ng  JL , Delaney  TA .  Cutaneous involvement by multiple myeloma presenting as erythematous indurated plaques at the site of cardiac pacemaker insertion.   JAAD Case Rep. 2021;12:54-57. doi:10.1016/j.jdcr.2021.04.013PubMedGoogle ScholarCrossref
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Noguchi  H , Matsumoto  T , Kimura  U , Hiruma  M , Kusuhara  M , Ihn  H .  Cutaneous cryptococcosis.   Med Mycol J. 2019;60(4):101-107. doi:10.3314/mmj.19.008PubMedGoogle ScholarCrossref
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Jordan  AA , Graciaa  DS , Gopalsamy  SN ,  et al.  Sweet syndrome imitating cutaneous cryptococcal disease.   Open Forum Infect Dis. 2022;9(11):ofac608. doi:10.1093/ofid/ofac608PubMedGoogle ScholarCrossref
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.

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