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A Woman With Purple Macules on the Legs

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

A 30-year-old woman presented with widespread, erythematous, purpuric macules and papules on the abdomen and extremities. The eruption had been present for approximately 8 months and consisted of asymptomatic, self-resolving, and continuously appearing macules and papules. Her medical history was significant for chronic hepatitis B infection, for which tenofovir was initiated 4 months ago. Review of systems was positive only for occasional arthralgias in her knees and ankles. She was not taking any other medications.

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Cutaneous small-vessel vasculitis (CSVV)

B. Evaluate for systemic vasculitis

The keys to the correct diagnosis are the physical examination and biopsy results. CSVV has a variable clinical appearance including purpuric papules, purpuric macules, urticaria-like lesions, petechiae, and ulcers.14 A distribution predominantly involving dependent areas in a nonretiform pattern is also clinically consistent with CSVV.1,2 In the case of suspected CSVV, it is important to establish the diagnosis and to assess for other end-organ involvement.

CSVV is a vasculitis involving postcapillary venules in the skin. The condition most commonly manifests as palpable purpura on the bilateral lower extremities. The incidence of CSVV is between 39.6 and 59.8 cases per million people annually.1 A skin biopsy is used to confirm the diagnosis, and histopathology shows leukocytoclastic vasculitis with extravasated erythrocytes, a neutrophilic infiltrate, fibrinoid necrosis, and broken-down neutrophils releasing nuclear debris (leukocytoclasis). A second biopsy should be obtained for direct immunofluorescence whenever possible, to evaluate for IgA-related CSVV. Importantly, for accurate direct immunofluorescence results, the newest lesion or a lesion that appeared within the past 24 to 48 hours should be sampled.

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Article Information

Corresponding Author: Roger S. Ho, MD, MS, MPH, The Ronald O. Perelman Department of Dermatology, New York University School of Medicine, 240 E 28th St, 11th Floor, New York, NY 10016 (Roger.Ho@nyumc.org).

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank the patient for providing permisison to share her information.

References
1.
Carlson  JA, Cavaliere  LF, Grant-Kels  JM.  Cutaneous vasculitis: diagnosis and management.  Clin Dermatol. 2006;24(5):414-429.PubMedGoogle ScholarCrossref
2.
Lotti  T, Ghersetich  I, Comacchi  C, Jorizzo  JL.  Cutaneous small-vessel vasculitis.  J Am Acad Dermatol. 1998;39(5, pt 1):667-687.PubMedGoogle ScholarCrossref
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Iglesias-Gamarra  A, Restrepo  JF, Matteson  EL.  Small-vessel vasculitis.  Curr Rheumatol Rep. 2007;9(4):304-311.PubMedGoogle ScholarCrossref
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Goeser  MR, Laniosz  V, Wetter  DA.  A practical approach to the diagnosis, evaluation, and management of cutaneous small-vessel vasculitis.  Am J Clin Dermatol. 2014;15(4):299-306.PubMedGoogle ScholarCrossref
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Sais  G, Vidaller  A, Jucglà  A, Servitje  O, Condom  E, Peyri  J.  Prognostic factors in leukocytoclastic vasculitis: a clinicopathologic study of 160 patients.  Arch Dermatol. 1998;134(3):309-315.PubMedGoogle ScholarCrossref
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Hochberg  MC.  Updating the American College of Rheumatology revised criteria for the classification of systemic lupus erythematosus.  Arthritis Rheum. 1997;40(9):1725.PubMedGoogle ScholarCrossref
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Lei  Y, Hu  T, Song  X,  et al.  Production of autoantibodies in chronic hepatitis B virus infection is associated with the augmented function of blood CXCR5+CD4+ T cells.  PLoS One. 2016;11(9):e0162241.PubMedGoogle ScholarCrossref
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Han  SH.  Extrahepatic manifestations of chronic hepatitis B.  Clin Liver Dis. 2004;8(2):403-418.PubMedGoogle ScholarCrossref
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Davis  P, Read  AE.  Antibodies to double-stranded (native) DNA in active chronic hepatitis.  Gut. 1975;16(6):413-415.PubMedGoogle ScholarCrossref
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Pop  TL, Stefănescu  A, Samaşca  G, Miu  N.  Clinical significance of the antinuclear antibodies in chronic viral hepatitis B in children.  Clin Lab. 2014;60(6):931-939.PubMedGoogle Scholar
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