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A 62-year-old woman with extranodal marginal zone lymphoma noticed small, asymptomatic, purpuric patches on her right buttock and bilateral lower extremities. This was accompanied by fever, night sweats, and fatigue. She was found to have clinical and radiologic progression of her lymphoma and began receiving R-CHOP (rituximab + cyclophosphamide + doxorubicin hydrochloride + vincristine [Oncovin] + prednisone) chemotherapy. Her purpuric patches improved initially after the first cycle of chemotherapy; however, in the weeks before the second cycle, they recurred more extensively, involving her right cheek and bilateral ears, arms, and legs. This time she also experienced pain in those areas as well as lower extremity edema.
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D. Test for serum cryoglobulin level
The key to the correct diagnosis is recognizing the subacute presentation of acral-predominant retiform purpura in a well-appearing patient with underlying lymphoma.
Retiform purpura can also present in ecthyma gangrenosum, a cutaneous syndrome that occurs when bacterial pathogens (commonly Pseudomonas aeruginosa) invade dermal vessels and cause neutrophilic cutaneous vasculitis.1 However, patients with ecthyma gangrenosum are usually septic and toxic-appearing, which is not the case in this patient. Patients with acute disseminated intravascular coagulation and cutaneous purpura are also generally ill-appearing and hemodynamically unstable. Some of them, especially those with the widespread purpura of purpura fulminans, can benefit from administration of protein C concentrate.2 However, disseminated intravascular coagulation is unlikely in this patient, given that she was otherwise well and her fibrinogen level and results of a coagulation panel were within normal limits. The calcium-phosphate product can be elevated in uremic calciphylaxis. However, extensive calciphylaxis typically involves fatty areas of the trunk and proximal extremities; involvement of the face and ears is atypical.
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Corresponding Author: Sotonye Imadojemu, MD, MBE, 221 Longwood Ave, Boston, MA 02115 (email@example.com).
Published Online: October 14, 2019. doi:10.1001/jama.2019.15276
Conflict of Interest Disclosures: None reported.
Funding/Support: Dr Li was supported by National Institutes of Health (NIH) grant T32GM007753.
Role of the Funder/Sponsor: The NIH had no role in patient care or in the concept, data acquisition, preparation, or the decision to submit the work for publication.
Additional Contributions: We thank the patient for providing permission to share her information.
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