Fatal varicella zoster virus infection as first manifestation of idiopathic CD4⁺ T-cell lymphocytopenia

Excerpt

Varicella zoster virus (VZV) is a neurotropic human alpha-herpes virus that causes varicella (chickenpox) initially and zoster upon reactivation of latent infection. In adults and immunocompromised patients, severe clinical complications can occur. In 1992, a new immunodeficiency syndrome called idiopathic CD4⁺ T-cell lymphocytopenia (ICL) was first described. ICL is characterized by a persistent CD4⁺ T-lymphocyte count of <300/μl or <20% of total T cells (measured on at least two occasions) in the absence of HIV infection and any other known immunodeficiency or therapy associated with lymphocytopenia. In affected patients, the immunoglobulin level may be normal or slightly decreased [1]. The clinical spectrum of infection ranges from an asymptomatic course or minimal symptoms to death due to various opportunistic infections. The following report describes a case of fatal progression of primary VZV infection in a patient with a post-mortem diagnosis of ICL. …