Nonbacterial purpura fulminans and severe autoimmune acquired protein S deficiency associated with human herpesvirus-6 active replication

Br J Dermatol. 2009 Jul;161(1):181-3. doi: 10.1111/j.1365-2133.2009.09264.x. Epub 2009 May 26.

Abstract

Nonbacterial purpura fulminans (PF) is rare, usually follows viral infection in young children, and is characterized by specific coagulation disorders, requiring specific therapy. Following a transient rash, a 2-year-old previously healthy girl developed PF without haemodynamic impairment. Laboratory data revealed disseminated intravascular coagulation and a severe transient protein S deficiency. Antiprotein S autoantibodies and active human herpesvirus-6 (HHV6) replication were demonstrated. Purpuric skin lesions spread very rapidly despite broad-spectrum antibiotics and right leg amputation. Plasmapheresis and intravenous immunoglobulins gave complete clinical recovery and normalization of protein S level within 10 days, with progressive clearance of antiprotein S autoantibodies. Transient severe protein S deficiencies have previously been reported in patients with nonbacterial PF, usually after varicella infection. This is the first documented case of PF after HHV6 infection.

Publication types

  • Case Reports

MeSH terms

  • Amputation, Surgical
  • Autoimmune Diseases / complications*
  • Autoimmune Diseases / therapy
  • Child, Preschool
  • Disseminated Intravascular Coagulation / etiology
  • Female
  • Heparin / therapeutic use
  • Herpesvirus 6, Human / isolation & purification
  • Herpesvirus 6, Human / physiology
  • Humans
  • Immunoglobulins / therapeutic use
  • Leg / surgery
  • Plasmapheresis / methods
  • Polymerase Chain Reaction
  • Protein S / analysis
  • Protein S Deficiency / etiology
  • Protein S Deficiency / therapy
  • Purpura Fulminans / therapy
  • Purpura Fulminans / virology*
  • Roseolovirus Infections / complications*
  • Treatment Outcome
  • Virus Replication

Substances

  • Immunoglobulins
  • Protein S
  • Heparin