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Red Plaques in a Pediatric Patient With Acute Leukemia of Ambiguous Lineage

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

A 16-year-old boy with acute leukemia of ambiguous lineage developed tender, 1- to 2-cm erythematous plaques and nodules during treatment with chemotherapy. Previously, he had been well, with morphological remission found on a recent bone marrow biopsy. The patient had no significant dermatological history, except an allergy to cashew nuts and a prior episode (6 mo) of urticaria during a platelet transfusion that had resolved with 1 dose of oral antihistamine. The patient’s medications included ondansetron, aprepitant, prednisolone, and oxycodone (supportive care initiated with chemotherapy), and long-term posaconazole and trimethoprim/sulfamethoxazole treatment.

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C. Neutrophilic eccrine hidradenitis

Results of the patient’s punch biopsy demonstrated a leukocytoclastic neutrophilic infiltrate in the deeper dermis, around the appendage structures and the coiled eccrine glands. This infiltrate spilled into the surrounding interstitium. No leukemic or infective cells were seen, and the epidermis and upper dermis were relatively unremarkable. These findings are consistent with neutrophilic eccrine hidradenitis (NEH).

A rare condition with characteristic histopathological findings, NEH has variable clinical features.1,2 Most often, NEH presents in patients undergoing cytotoxic chemotherapy for hematological malignant neoplasms, particularly acute myeloid leukemia.2 Although cytarabine is considered to be the most common causative agent, other cytotoxic medications, including daunorubicin, decitabine, BRAF inhibitors, cetuximab, and imatinib, have been described as causing NEH.1,2 Non-antineoplastic agents described as causative include acetaminophen, adalimumab, azathioprine, and G-CSF, among others.1 Neutrophilic eccrine hidradenitis has been observed in other clinical settings, affecting patients with HIV, solid organ malignant neoplasms, and infections; among pediatric patients, it can occur without a precipitating event.1,3,4

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Article Information

Corresponding Author: Arabella Wallett, MBBS, Women’s and Children’s Hospital, South Australia, 72 King William St, North Adelaide, Adelaide, South Australia 5006, Australia (arabella.wallett@sa.gov.au).

Published Online: December 30, 2020. doi:10.1001/jamaoncol.2020.6134

Conflict of Interest Disclosures: None reported.

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

References
1.
Nelson  CA , Stephen  S , Ashchyan  HJ , James  WD , Micheletti  RG , Rosenbach  M .  Neutrophilic dermatoses: pathogenesis, Sweet syndrome, neutrophilic eccrine hidradenitis, and Behçet disease.   J Am Acad Dermatol. 2018;79(6):987-1006. doi:10.1016/j.jaad.2017.11.064PubMedGoogle ScholarCrossref
2.
Bachmeyer  C , Aractingi  S .  Neutrophilic eccrine hidradenitis.   Clin Dermatol. 2000;18(3):319-330. doi:10.1016/S0738-081X(99)00123-6PubMedGoogle ScholarCrossref
3.
Bassas-Vila  J , Fernández-Figueras  MT , Romaní  J , Ferrándiz  C .  Infectious eccrine hidradenitis: a report of 3 cases and a review of the literature.   Actas Dermosifiliogr. 2014;105(2):e7-e12. doi:10.1016/j.ad.2013.04.016PubMedGoogle ScholarCrossref
4.
Shih  IH , Huang  YH , Yang  CH , Yang  LC , Hong  HS .  Childhood neutrophilic eccrine hidradenitis: a clinicopathologic and immunohistochemical study of 10 patients.   J Am Acad Dermatol. 2005;52(6):963-966. doi:10.1016/j.jaad.2005.01.009PubMedGoogle ScholarCrossref
5.
Shear  NH , Knowles  SR , Shapiro  L , Poldre  P .  Dapsone in prevention of recurrent neutrophilic eccrine hidradenitis.   J Am Acad Dermatol. 1996;35(5 pt 2):819-822. doi:10.1016/S0190-9622(96)90092-4PubMedGoogle ScholarCrossref
6.
Martínez-Leboráns  L , Victoria-Martínez  AM , Torregrosa-Calatayud  JL , Alegre de Miquel  V .  Leucemia cutis. Serie de 17 casos y revisión de la literatura.   Actas Dermosifiliogr. 2016;107(9):e65-e69. doi:10.1016/j.ad.2016.02.015PubMedGoogle ScholarCrossref
7.
Patterson  J.   Weedon’s Skin Pathology. 4th ed. Elsevier Health Sciences; 2015.
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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