Nonbacterial purpura fulminans and severe autoimmune acquired protein S deficiency associated with human herpesvirus-6 active replication.
Br J Dermatol
; 161(1): 181-3, 2009 Jul.
Article
em En
| MEDLINE
| ID: mdl-19545295
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.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Doenças Autoimunes
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Infecções por Roseolovirus
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Púrpura Fulminante
Tipo de estudo:
Etiology_studies
/
Risk_factors_studies
Limite:
Child, preschool
/
Female
/
Humans
Idioma:
En
Ano de publicação:
2009
Tipo de documento:
Article