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[Pustular vasculitis disclosing Behçet disease]

J Castanet1, S Carmignani, J P Lacour

  • 1Service de Dermatologie, Hôpital Pasteur, Nice.

Annales De Dermatologie Et De Venereologie
|January 1, 1994
PubMed
Summary
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Pustular vasculitis, a Behçet's disease symptom, can present with unique arch-shaped lesions. This case highlights lymphocytic infiltration in histology and effective colchicine treatment for skin relapses.

Area of Science:

  • Dermatology
  • Rheumatology
  • Immunology

Background:

  • Pustular vasculitis is a recognized manifestation of Behçet's disease.
  • Behçet's disease is a multisystem inflammatory disorder characterized by recurrent oral aphthae, genital ulcers, skin lesions, and ocular inflammation.
  • The case details a patient diagnosed with Behçet's disease based on specific clinical criteria.

Observation:

  • A 29-year-old patient presented with recurrent, episodic papulopustular skin eruptions forming an unusual arch pattern.
  • Histological examination of the skin lesions revealed a predominant lymphocytic infiltration.
  • The patient had a history of recurrent oral aphthosis, accompanied by lymphocytic meningitis, uveitis, and erythema nodosum.

Findings:

  • The patient's papulopustular eruption exhibited a distinct arch formation.

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  • Histopathology demonstrated a predominantly lymphocytic infiltrate, contrasting with the typical polynuclear infiltration seen in pustular vasculitis.
  • While corticosteroids were initially effective, they did not prevent relapses, necessitating alternative treatment.

    • Implications:

    • The findings suggest that predominant lymphocytic infiltration in pustular vasculitis may be a specific indicator for Behçet's disease, potentially linked to a pathergy phenomenon.
    • Colchicine proved effective in managing skin relapses, although the precise mechanism of action in this context requires further elucidation.
    • This case expands the understanding of pustular vasculitis presentations and treatment responses in Behçet's disease.