Fatal Chronic Varicella-Zoster Viral Infection in a Young Man With Chediak-Higashi Syndrome

Albane Badet; Clément Pruvot; Zoé Manssens; Wadih Abou-Chahla; Sébastien Buche

doi:10.1111/pde.70082

Fatal Chronic Varicella-Zoster Viral Infection in a Young Man With Chediak-Higashi Syndrome

Pediatr Dermatol. 2025 Nov 20. doi: 10.1111/pde.70082. Online ahead of print.

Authors

Albane Badet^{1

2}, Clément Pruvot^{1

2

3}, Zoé Manssens⁴, Wadih Abou-Chahla⁵, Sébastien Buche^{1

3}

Affiliations

¹ Department of Dermatology, Lille University Hospital, Lille, France.
² University of Lille, Lille, France.
³ Pediatric Dermatology Department, Lille University Hospital, Lille, France.
⁴ Pathology Department, Lille University Hospital, Lille, France.
⁵ Pediatric Hematology and Oncology Department, Jeanne de Flandre Hospital, Lille University Hospital, Lille, France.

PMID: 41262052
DOI: 10.1111/pde.70082

Abstract

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive primary immunodeficiency characterized by partial oculocutaneous albinism, neurologic involvement, and a predisposition to severe infections. Patients are particularly susceptible to developing hemophagocytic lymphohistiocytosis (HLH), which significantly worsens prognosis. We report the case of a 19-year-old male with CHS, under long-term immunosuppressive therapy for chronic HLH, who developed chronic VZV infection. Despite treatment with valacyclovir, the lesions progressed, and virological investigations confirmed the diagnosis of acyclovir-resistant varicella-zoster virus (VZV). The clinical course was marked by the appearance of neurological symptoms and a fatal outcome. To our knowledge, this is the first reported fatal case of chronic VZV infection with systemic dissemination in a patient with CHS. This case highlights the risk of resistance associated with long-term prophylaxis and the complexity of managing herpesvirus infections in immunocompromised patients receiving JAK inhibitors.

Keywords: Chediak–Higashi syndrome; JAK inhibitors; acyclovir resistance; chronic VZV infection; herpesviruses; immunodeficiency; macrophage activation syndrome.

Publication types

Case Reports