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Bright Red Macules of the Lower Legs

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

A woman in her 70s was referred to the dermatology department by the rheumatology department with concerns for vasculitis of the bilateral lower legs. She had been healthy until 6 years earlier when she had suddenly developed peripheral neuropathy, with subsequent workup revealing a diagnosis of immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance. Her symptoms had rapidly progressed during the past few months. In addition to worsening neuropathy, she had developed fatigue, weight loss, and sudden altered mental status. An ischemic stroke was identified. The patient was hospitalized, and extensive diagnostic examinations and procedures were completed. A lymphoproliferative disorder with plasmocytic differentiation was detected in a bone marrow biopsy specimen, which together with the monoclonal IgM paraproteinemia was consistent with Waldenström macroglobulinemia (WM).

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C. Cutaneous macroglobulinosis

Tests results of serum viscosity, cryoglobulins, and cold agglutinins returned normal. Histopathologic findings revealed many amorphous eosinophilic deposits strongly positive for periodic acid–Schiff stain (PAS) in the papillary dermis. Congo red stain result was negative, with no apple-green birefringence. Immunofluorescence showed strong reactivity for IgM and lambda chains. Overall, these findings were consistent with cutaneous macroglobulinosis (CM).

Waldenström macroglobulinemia is a lymphoplasmacytic lymphoma associated with IgM hypersecretion and invasion of neoplastic B cells in bone marrow and peripheral organs.1 It most commonly affects people from 60 to 70 years of age. One-quarter of patients are asymptomatic at diagnosis; however, 40% of individuals will experience symptoms within 3 years.1 The most commonly associated systemic manifestations include lymphadenopathy, anemia, and hepatosplenomegaly.1,2 Neuropathy, which the patient in this case experienced, is present in as many as one-third of individuals. Interestingly, this prevalence seems higher in patients with CM.3,4

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Article Information

Corresponding Authors: Avery H. LaChance, MD, MPH, and Michelle S. Min, MD, MS, Department of Dermatology, Brigham and Women’s Hospital, Harvard Medical School, 221 Longwood Ave, Boston, MA 02215 (alachance@bwh.harvard.edu; michellesmin@gmail.com).

Published Online: August 11, 2021. doi:10.1001/jamadermatol.2021.2980

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting us permission to publish this information. We also thank Camilla Villa-Ruiz, MD, MPH (Medical Sciences, University of Puerto Rico) for data interpretation; and Eleanor Russell-Goldman, MD, PhD, MBBCH, and Brian Quattrochi, MD, PhD (Department of Pathology, Brigham and Women's Hospital) for their dermatopathological expertise and assistance with diagnosing this unique case.

Author Contributions: Drs Min and LaChance contributed equally as senior authors of this work.

References
1.
Advani  P , Paulus  A , Ailawadhi  S .  Updates in prognostication and treatment of Waldenström’s macroglobulinemia.   Hematol Oncol Stem Cell Ther. 2019;12(4):179-188. doi:10.1016/j.hemonc.2019.05.002 PubMedGoogle ScholarCrossref
2.
Libow  LF , Mawhinney  JP , Bessinger  GT .  Cutaneous Waldenström’s macroglobulinemia: report of a case and overview of the spectrum of cutaneous disease.   J Am Acad Dermatol. 2001;45(6)(suppl):S202-S206. doi:10.1067/mjd.2001.103262 PubMedGoogle ScholarCrossref
3.
Gressier  L , Hotz  C , Lelièvre  JD ,  et al.  Cutaneous macroglobulinosis: a report of 2 cases.   Arch Dermatol. 2010;146(2):165-169. doi:10.1001/archdermatol.2009.359 PubMedGoogle ScholarCrossref
4.
Klein  CJ , Moon  JS , Mauermann  ML ,  et al.  The neuropathies of Waldenström’s macroglobulinemia (WM) and IgM-MGUS.   Can J Neurol Sci. 2011;38(2):289-295. doi:10.1017/S0317167100011483 PubMedGoogle ScholarCrossref
5.
Hassab-El-Naby  HMM , El-Khalawany  M , Rageh  MA .  Cutaneous macroglobulinosis with Waldenström macroglobulinemia.   JAAD Case Rep. 2020;6(8):771-775. doi:10.1016/j.jdcr.2020.06.024PubMedGoogle ScholarCrossref
6.
Camp  BJ , Magro  CM .  Cutaneous macroglobulinosis: a case series.   J Cutan Pathol. 2012;39(10):962-970. doi:10.1111/j.1600-0560.2012.01983.x PubMedGoogle ScholarCrossref
7.
Shiba  K , Abe  R , Miyauchi  T , Nomura  T , Kondo  T , Shimizu  H .  Massive petechiae as an initial symptom of Waldenström’s macroglobulinemia.   Int J Dermatol. 2016;55(6):e361-e362. doi:10.1111/ijd.13189 PubMedGoogle ScholarCrossref
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Rafiei  R , Eftekhari  H , Rafiee  B .  Cutaneous amyloidosis as the first presentation of Waldenstrom macroglobulinemia.   Caspian J Intern Med. 2020;11(3):340-342. doi:10.22088/cjim.11.3.340PubMedGoogle Scholar
9.
Cohen  SJ , Pittelkow  MR , Su  WP .  Cutaneous manifestations of cryoglobulinemia: clinical and histopathologic study of seventy-two patients.   J Am Acad Dermatol. 1991;25(1 Pt 1):21-27. doi:10.1016/0190-9622(91)70168-2 PubMedGoogle Scholar
10.
Brenn  T , McKee  TM . Vascular diseases, superficial and deep perivascular inflammatory dermatoses. In: McKee  PH , Calonje  E , Granter  SR ,  et al.  Pathology of the Skin. 3rd ed. Elsevier; 2005:277-278, 709-710.
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