Nonbacterial purpura fulminans and severe autoimmune acquired protein S deficiency associated with human herpesvirus-6 active replication.
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.
Boccara
O
O
Department of Dermatology, University René DescartesParis V, 75473 Paris cedex 15, France. oliveboc@yahoo.com
Lesage
F
F
Regnault
V
V
Lasne
D
D
Dupic
L
L
Bourdon-Lanoy
E
E
Pannier
S
S
Fraitag
S
S
Audat
F
F
Lecompte
T
T
Hubert
P
P
Bodemer
C
C
D000671:Amputation, Surgical; D001327:Autoimmune Diseases / Q000150:complications* / Q000628:therapy; D002675:Child, Preschool; D004211:Disseminated Intravascular Coagulation / Q000209:etiology; D005260:Female; D006493:Heparin / Q000627:therapeutic use; D015654:Herpesvirus 6, Human / Q000302:isolation & purification / Q000502:physiology; D006801:Humans; D007136:Immunoglobulins / Q000627:therapeutic use; D007866:Leg / Q000601:surgery; D010956:Plasmapheresis / Q000379:methods; D016133:Polymerase Chain Reaction; D017293:Protein S / Q000032:analysis; D018455:Protein S Deficiency / Q000209:etiology / Q000628:therapy; D055665:Purpura Fulminans / Q000628:therapy / Q000821:virology*; D019349:Roseolovirus Infections / Q000150:complications*; D016896:Treatment Outcome; D014779:Virus Replication
D007136:Immunoglobulins; D017293:Protein S; D006493:Heparin