Atypical varicella zoster infection associated with hemophagocytic lymphohistiocytosis.
Two adolescents, on immunosuppressive therapy for graft-versus-host disease, developed hemophagocytic lymphohistiocytosis (HLH) after varicella zoster virus (VZV) reactivation. In the absence of dermatome restricted characteristic skin lesions, VZV reactivation was not immediately recognized and treatment with acyclovir was delayed. The first patient developed optical neuritis and died 2 months after the VZV episode due to massive intracranial hemorrhage. The second patient presented with severe abdominal pain and pancreatitis, followed by atypical skin eruptions, which prompted a faster diagnosis. Both patients recovered from their HLH, the first patient being successfully treated with immunosuppressive agents and the second with VZV treatment only. These two cases demonstrate the difficulties in recognizing VZV reactivation, and in order to start adequate and timely treatment, the need to consider VZV as a possible cause of HLH in severely immunocompromised patients.
Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands. jvdwerff@uzbrussel.be
Jutte E
JE
van der Werff ten Bosch
Wouter J W
WJ
Kollen
Lynne M
LM
Ball
Danielle M C
DM
Brinkman
Anne C T M
AC
Vossen
Arjan C
AC
Lankester
R Maarten
RM
Egeler
Robbert G M
RG
Bredius
D000212:Acyclovir / Q000627:therapeutic use; D000293:Adolescent; D000998:Antiviral Agents / Q000627:therapeutic use; D006086:Graft vs Host Disease / Q000188:drug therapy; D006562:Herpes Zoster / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D016867:Immunocompromised Host; D015470:Leukemia, Myeloid, Acute / Q000628:therapy; D051359:Lymphohistiocytosis, Hemophagocytic / Q000276:immunology* / Q000503:physiopathology / Q000821:virology*; D008297:Male
D000998:Antiviral Agents; D000212:Acyclovir