Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Melissa Nickles; Samantha Hunt; Saul Turcios-Escobar; Amaara Babwah; Nisreen Mobayed; Carlos Murga-Zamalloa; Michelle Bain; John Quigley; Paul Rubinstein; Carlos Galvez

doi:10.1097/DAD.0000000000002720

Richter Syndrome Presenting as Subcutaneous Nodules and a Dermal Plaque

Am J Dermatopathol. 2024 Apr 23. doi: 10.1097/DAD.0000000000002720. Online ahead of print.

Affiliations

¹ Department of Dermatology, University of Illinois, Chicago, IL.
² Department of Pathology, University of Illinois, Chicago, IL.
³ Department of Hematology, University of Illinois, Chicago, IL; and.
⁴ Department of Internal Medicine, University of Illinois, Chicago, IL.

PMID: 38648045
DOI: 10.1097/DAD.0000000000002720

Abstract

Richter syndrome (RS) describes a phenomenon in which a patient with chronic lymphocytic leukemia (CLL) develops an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL). Reports of cutaneous RS remain exceedingly rare. We report a 61-year-old woman with relapsed/refractory CLL presenting with several subcutaneous nodules on her arms and legs and a single dermal plaque on her abdomen. Skin biopsy revealed a diagnosis of DLBCL, ABC-type, and her clinical status rapidly deteriorated following diagnosis. We review the variety of clinical presentations of cutaneous RS, its association with CLL, risk factors for RS development in CLL patients, and the distinctive histopathologic and immunophenotypic features of DLBCL. We hope to highlight the importance of prompt skin biopsy in patients with CLL presenting with progressive skin lesions and increase awareness of this aggressive clinical syndrome.