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Systemic inflammatory response syndrome associated with Sweet's syndrome

Enrique Otheo1, Purificación Ros, José L. Vázquez

  • 1Pediatric Intensive Care Unit (EO, PR, JLV, IM), Pathology Department, Dermatology Department, and Pediatric Hematology-Oncology Unit, Hospital Universitario Ramón y Cajal, Alcalá de Henares University, Madrid, Spain.

Pediatric Critical Care Medicine : a Journal of the Society of Critical Care Medicine and the World Federation of Pediatric Intensive and Critical Care Societies
|June 5, 2003
PubMed

Insights

Sweet's syndrome can lead to severe systemic inflammatory response syndrome, septic shock, and multiple organ dysfunction syndrome in children. Prompt steroid therapy is crucial for recovery in these rare cases.

Area of Science:

  • Pediatric Hematology-Oncology
  • Dermatology
  • Critical Care Medicine

Background:

  • Sweet's syndrome is an acute febrile neutrophilic dermatosis.
  • It is rarely associated with systemic inflammatory conditions.
  • Pediatric cases are exceptionally uncommon.

Purpose of the Study:

  • To report the first pediatric case of Sweet's syndrome complicated by systemic inflammatory response syndrome (SIRS), septic shock, and multiple organ dysfunction syndrome (MODS).
  • To highlight the diagnostic and therapeutic challenges in pediatric patients with these co-occurring conditions.

Main Methods:

  • Case report of a 7-year-old female patient post-autologous bone marrow transplantation for acute nonlymphoblastic leukemia.
  • Clinical presentation included characteristic skin lesions and fever, meeting Sweet's syndrome criteria.
  • Management involved broad-spectrum antimicrobial therapy, cardiovascular support, and subsequent steroid administration.

Main Results:

  • The patient developed SIRS, septic shock, and MODS despite initial treatments.
  • Infectious sources were ruled out by negative cultures.
  • Dramatic clinical improvement was observed following systemic steroid therapy, leading to full recovery.

Conclusions:

  • Sweet's syndrome should be considered in pediatric patients presenting with typical skin lesions and SIRS, even post-transplant.
  • Early recognition and initiation of systemic steroid therapy are critical for favorable outcomes in such rare presentations.
  • This case underscores the potential severity of Sweet's syndrome and the efficacy of steroid treatment.

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