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Granulocyte-dependent Autoantibody-induced Skin Blistering
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Published on: October 12, 2012

[Cutaneous polyarteritis nodosa--is it really benign?].

Eran Shavit1, Amir Horev, Eitan Berman

  • 1Department of Dermatology and Venereology, Soroka Medical Center, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel. eransha@clalit.org.il

Harefuah
|January 16, 2013
PubMed
Summary

Cutaneous polyarteritis nodosa (PAN), usually benign, can present aggressively. This case highlights a severe, progressive form leading to amputation and death despite treatment.

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Area of Science:

  • Vascular Medicine
  • Dermatology
  • Rheumatology

Background:

  • Polyarteritis nodosa (PAN) is a systemic necrotizing vasculitis affecting medium arteries.
  • Cutaneous PAN, a limited form, typically follows a benign, chronic course.
  • Diabetes mellitus is a common comorbidity in patients with vasculitic conditions.

Observation:

  • A 47-year-old female with diabetes presented with painful leg ulcers.
  • Clinical and histological findings confirmed PAN, limited to the skin.
  • Despite corticosteroids and azathioprine, ulcers progressed with extensive necrosis.

Findings:

  • The patient required bilateral above-knee amputations.
  • The disease course was unusually severe and progressive.
  • Fatal outcome occurred within three years of diagnosis.

Implications:

  • This case challenges the assumption of a benign course for cutaneous PAN.
  • Aggressive, treatment-refractory cutaneous PAN can lead to devastating outcomes.
  • Highlights the need for vigilant monitoring and potentially novel therapeutic strategies in severe cutaneous PAN.