Successful Interferon-α Therapy for a Huge Lesion Including Eye and Eyelids

Introduction

Defective T cell responses lead to insufficient interferon production, thereby compromising antiviral immunity and increasing vulnerability to viral infections in combined immunodeficiency disorders. The DOCK8 deficiency, also known as autosomal recessive (AR) hyper IgE syndrome (HIES), is a combined immunodeficiency characterized by susceptibility to eczema, respiratory tract infections, allergies, malignancies, autoimmunity, and cutaneous viral infections caused by herpes simplex virus, pox virus, human papillomavirus, molluscum contagiosum virus, and orf virus, along with elevated serum IgE levels and hypereosinophilia (Goker E et al., Pediatr Allergy Immunol 2022; 33: e13666).

DOCK8 encodes a guanine nucleotide exchange factor that regulates the actin cytoskeletonʼs response to mitogenic and chemokine signals via the reversible activation of small G proteins, including cell division cycle 42 (Harada Y et al., Blood 2012; 119: 4451–4461). DOCK8 deficiency affects the migration, function, and viability of both innate and adaptive immune cells. The memory compartments of T and B cells, lymphoproliferation in response to mitogens, T cell migration, and NK cell cytotoxicity are predominantly diminished (Zhang Q et al., N Engl J Med 2009; 361: 2046–2055). Patients with DOCK8 deficiency suffer persistent cutaneous viral infections, potentially due to compromised actin cytoskeleton responses, which are crucial for immune cell migration to infection sites, and a reduction in plasmacytoid dendritic cells (pDC) that secrete essential type 1 interferons for viral clearance (Mizesko M et al., J Allergy Clin Immunol 2013; 131: 840–848). Type I interferons (IFNs) upregulate the expression of major histocompatibility complex molecules I and II, enhancing the presentation of viral peptides to cytotoxic T cells through conventional DCs, as well advocating NK functions. Both non-pegylated and pegylated IFN-α (PEG IFN-a) were used successful for difficult-to-treat cutaneous viral infections in a few patients with HIES (Al-Zahrani D et al., Clin Immunol 2014; 153: 104–108; Papan C et al., J Allergy Clin Immunol 2014; 133: 1456–1458; Kilic S et al., Pediatrics 2006; 117: e1253–e1255; Benninghoff U et al., Pediatr Allergy Immunol 2008; 19: 564–568).

Publication History

Received: 22 May 2025

Accepted after revision: 05 November 2025

Article published online:
09 December 2025