Fatal cytophagic histiocytic panniculitis

Pediatr Dermatol. 2004 May-Jun;21(3):246-9. doi: 10.1111/j.0736-8046.2004.21312.x.

Abstract

Individual cases of so-called Weber-Christian disease with a bleeding diathesis have been reported for several years. These were originally diagnosed as Weber-Christian disease, but have been recategorized on review as a chronic, visceral, and cutaneous histiocytic (cytophagic) panniculitis, progressing to liver dysfunction and jaundice and a terminal hemorrhagic diathesis. We report here a rare catastrophic form of systemic panniculitis in an adolescent girl. Despite compelling clinical evidence, the diagnosis was made only on postmortem biopsies.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Fatal Outcome
  • Female
  • Humans
  • Panniculitis, Nodular Nonsuppurative / pathology*