[Skip to Content]
[Skip to Content Landing]

Ecchymotic Plaques on the Scalp of a Man in His 80s

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 80s presented to the emergency department with a 4-week history of progressive weakness and fatigue, with associated development of purple bruiselike lesions on his head. He denied any history of trauma, falls, or occlusive headgear use. His medical history was significant for atrial fibrillation, receiving anticoagulation medication, and heart failure.

On physical examination, numerous nontender violaceous plaques were seen on the forehead and scalp of the patient (Figure, A). On full skin examination, nonspecific skin-colored plaques were noted on the chest, and a 1.8 × 2.5-cm erythematous plaque was noted on the left lower back. There was no appreciable lymphadenopathy or hepatosplenomegaly. Peripheral blood test results revealed a hemoglobin level of 9.1 g/dL, a platelet count of 68 × 103/μL, a white blood cell count of 2800/μL, and an absolute neutrophil count of 1.0/μL. Lactate dehydrogenase level was 202 U/L. (To convert hemoglobin to g/L, multiply by 10.0; platelets to ×109/L, by 1; white blood cell and neutrophil counts to ×109/L, by 0.001; and lactate dehydrogenase to μkat/L, by 0.0167.) A 4-mm punch biopsy was performed on the erythematous plaque on the left lower back (Figure, B and C).

Please finish quiz first before checking answer.

You answered correctly!

Read the answer below and download your certificate.

You answered incorrectly.

Read the discussion below and retake the quiz.

B. Blastic plasmacytoid dendritic cell neoplasm

Histological assessment of skin biopsy revealed superficial and middermal infiltrate of small to medium basophilic blastoid cells, extending into the subcutaneous tissue and sparing epidermis with a grenz zone. Immunohistochemistry was positive for CD123 (Figure, D), CD4, CD56, CD7, and CD45, whereas myeloperoxidase was negative. The MIB-1 proliferation index was elevated at 70%. Overall, these findings were consistent with cutaneous blastic plasmacytoid dendritic cell neoplasm (BPDCN). A bone marrow biopsy was subsequently performed; this revealed hypercellular marrow for age, with estimated cellularity of 90%. Most cells were medium-large blasts that were positive for CD4, CD56, CD68, and CD123 and negative for CD34 and CD117. Hematologists noted the profile, occurring in the absence of myeloid and lymphoid lineage specific markers, as being effectively pathognomonic BPDCN.

Survey Complete!

Sign in to take quiz and track your certificates

Buy This Activity
Our websites may be periodically unavailable between 7:00pm CT June 10, 2023 and 1:00am CT June 11, 2023 for regularly scheduled maintenance.

JN Learning™ is the home for CME and MOC from the JAMA Network. Search by specialty or US state and earn AMA PRA Category 1 Credit(s)™ from articles, audio, Clinical Challenges and more. Learn more about CME/MOC

CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.

Article Information

Corresponding Author: Sophie Diong, MB BCh BAO (Hons), Dermatology Department, St James’s Hospital, James St, Dublin D08 NHY1, Ireland (sophiediong@gmail.com).

Published Online: May 24, 2023. doi:10.1001/jamadermatol.2023.1083

Conflict of Interest Disclosures: None reported.

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

References
1.
Economides  MP , Konopleva  M , Pemmaraju  N .  Recent developments in the treatment of blastic plasmacytoid dendritic cell neoplasm.   Ther Adv Hematol. 2019;10:2040620719874733. doi:10.1177/2040620719874733 PubMedGoogle ScholarCrossref
2.
Khoury  JD , Solary  E , Abla  O ,  et al.  The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms.   Leukemia. 2022;36(7):1703-1719. doi:10.1038/s41375-022-01613-1 PubMedGoogle ScholarCrossref
3.
Poussard  M , Angelot-Delettre  F , Deconinck  E .  Conventional therapeutics in BPDCN patients—do they still have a place in the era of targeted therapies?   Cancers (Basel). 2022;14(15):3767. doi:10.3390/cancers14153767 PubMedGoogle ScholarCrossref
4.
Pemmaraju  N , Konopleva  M .  Approval of tagraxofusp-erzs for blastic plasmacytoid dendritic cell neoplasm.   Blood Adv. 2020;4(16):4020-4027. doi:10.1182/bloodadvances.2019000173 PubMedGoogle ScholarCrossref
5.
Hirner  JP , O’Malley  JT , LeBoeuf  NR .  Blastic plasmacytoid dendritic cell neoplasm: the dermatologist’s perspective.   Hematol Oncol Clin North Am. 2020;34(3):501-509. doi:10.1016/j.hoc.2020.01.001 PubMedGoogle ScholarCrossref
6.
Saini  V , Marchese  A , Majetschak  M .  CXC chemokine receptor 4 is a cell surface receptor for extracellular ubiquitin.   J Biol Chem. 2010;285(20):15566-15576. doi:10.1074/jbc.M110.103408 PubMedGoogle ScholarCrossref
7.
Brüggen  MC , Valencak  J , Stranzenbach  R ,  et al.  Clinical diversity and treatment approaches to blastic plasmacytoid dendritic cell neoplasm: a retrospective multicentre study.   J Eur Acad Dermatol Venereol. 2020;34(7):1489-1495. doi:10.1111/jdv.16215 PubMedGoogle ScholarCrossref
8.
Sullivan  JM , Rizzieri  DA .  Treatment of blastic plasmacytoid dendritic cell neoplasm.   Hematology Am Soc Hematol Educ Program. 2016;2016(1):16-23. doi:10.1182/asheducation-2016.1.16 PubMedGoogle ScholarCrossref
9.
Sirohi  D , Smith  SC , Agarwal  N , Maughan  BL .  Unclassified renal cell carcinoma: diagnostic difficulties and treatment modalities.   Res Rep Urol. 2018;10:205-217. doi:10.2147/RRU.S154932 PubMedGoogle ScholarCrossref
10.
Hornick  JL .  Practical Soft Tissue Pathology: A Diagnostic Approach. 2nd ed. Elsevier; 2019.
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.

Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
Want full access to the AMA Ed Hub?
After you sign up for AMA Membership, make sure you sign in or create a Physician account with the AMA in order to access all learning activities on the AMA Ed Hub
Buy this activity
Close
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Education Center Collection Sign In Modal Right
Close

Name Your Search

Save Search
With a personal account, you can:
  • Access free activities and track your credits
  • Personalize content alerts
  • Customize your interests
  • Fully personalize your learning experience
Close
Close

Lookup An Activity

or

My Saved Searches

You currently have no searches saved.

Close

My Saved Courses

You currently have no courses saved.

Close