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Relationship between cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA): Blinded

Amandine Buffiere-Morgado1, Maxime Battistella1, Marie-Dominique Vignon-Pennamen1

  • 1University Paris Diderot, Sorbonne Paris Cité, Assistance Publique des Hôpitaux de Paris (AP-HP), Dermatology, Pathology, and Internal Medicine Department, Saint Louis Hospital, Paris, France.

Journal of the American Academy of Dermatology
|October 15, 2015
PubMed
Abstract

Insights

Cutaneous polyarteritis nodosa (cPAN) and macular lymphocytic arteritis (MLA) may not be distinct conditions. This study found overlapping clinical and histological features, suggesting a single disease spectrum for these vasculitides.

Area of Science:

  • Dermatology
  • Rheumatology
  • Pathology

Background:

  • Cutaneous polyarteritis nodosa (cPAN) is a medium vessel vasculitis presenting with livedo, nodules, and ulcerations.
  • Macular lymphocytic arteritis (MLA) is a small vessel vasculitis characterized by macules and specific histological findings.
  • Distinguishing between cPAN and MLA has been challenging due to overlapping features.

Purpose of the Study:

  • To evaluate the frequency of clinical and histological features of MLA in patients diagnosed with cPAN.
  • To determine if cPAN and MLA represent distinct disease entities.

Main Methods:

  • A monocentric retrospective analysis was conducted.
  • Skin biopsy specimens from patients diagnosed with cPAN were assessed by blinded pathologists.
  • 35 patients with cPAN were included in the study.

Main Results:

  • All patients presented with infiltrated livedo, nodules, or both; ulceration was infrequent.
  • Erythematous or pigmented lesions, characteristic of MLA, were observed in 54% of cPAN patients.
  • Histological findings such as lymphocytic arteritis (60%), concentric fibrin ring (18%), and absence of internal elastic lamina disruption (23%) were noted in cPAN biopsies.
  • Neutrophil paucity was present in 20% of cases.
  • No systemic involvement was observed, with a 57% complete remission rate over a median follow-up of 11 years.

Conclusions:

  • The study identified significant overlap in clinical and histological features between cPAN and MLA.
  • The findings suggest that cPAN and MLA may not be separate entities but rather part of a disease spectrum.
  • Further research, including studies with control groups, is needed to definitively classify these conditions.
Keywords:
cutaneous periarteritis nodosalymphocytic thrombophilic arteritismacular arteritismacular lymphocytic arteritisperiarteritis nodosavasculitis

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