Fatal cytophagic histiocytic panniculitis

Secmeer G., Sakalli H., Gok F., Ozen S., Kara A., Cengiz A., ...More

PEDIATRIC DERMATOLOGY, vol.21, no.3, pp.246-249, 2004 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 21 Issue: 3
  • Publication Date: 2004
  • Doi Number: 10.1111/j.0736-8046.2004.21312.x
  • Journal Name: PEDIATRIC DERMATOLOGY
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.246-249
  • Hacettepe University Affiliated: Yes

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.