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A Patient With Widespread Painful Purpura

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

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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Lymphoma-associated cryoglobulinemia

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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Article Information

Corresponding Author: Sotonye Imadojemu, MD, MBE, 221 Longwood Ave, Boston, MA 02115 (simadojemu@bwh.harvard.edu).

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.

References
1.
Somer  T, Finegold  SM.  Vasculitides associated with infections, immunization, and antimicrobial drugs.  Clin Infect Dis. 1995;20(4):1010-1036. doi:10.1093/clinids/20.4.1010PubMedGoogle ScholarCrossref
2.
Schellongowski  P, Bauer  E, Holzinger  U,  et al.  Treatment of adult patients with sepsis-induced coagulopathy and purpura fulminans using a plasma-derived protein C concentrate (Ceprotin).  Vox Sang. 2006;90(4):294-301. doi:10.1111/j.1423-0410.2006.00760.xPubMedGoogle ScholarCrossref
3.
Roccatello  D, Saadoun  D, Ramos-Casals  M,  et al.  Cryoglobulinaemia.  Nat Rev Dis Primers. 2018;4(1):11. doi:10.1038/s41572-018-0009-4PubMedGoogle ScholarCrossref
4.
Terrier  B, Karras  A, Kahn  JE,  et al.  The spectrum of type I cryoglobulinemia vasculitis: new insights based on 64 cases.  Medicine (Baltimore). 2013;92(2):61-68. doi:10.1097/MD.0b013e318288925cPubMedGoogle ScholarCrossref
5.
Cacoub  P, Comarmond  C, Domont  F, Savey  L, Saadoun  D.  Cryoglobulinemia vasculitis.  Am J Med. 2015;128(9):950-955. doi:10.1016/j.amjmed.2015.02.017PubMedGoogle ScholarCrossref
6.
Wysong  A, Venkatesan  P.  An approach to the patient with retiform purpura.  Dermatol Ther. 2011;24(2):151-172. doi:10.1111/j.1529-8019.2011.01392.xPubMedGoogle ScholarCrossref
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