Epidermodysplasia Verruciformis Revealing a Late Onset Combined Immunodeficiency (LOCID): An Unusual Case

Abstract

We report a rare association of Late Onset Combined Immunodeficiency (LOCID) with epidermodysplasia verruciformis (EV) in a 23-year-old Moroccan woman. At age 7, the patient developed thousands of small verrucae and experienced recurrent respiratory and gastrointestinal infections. A skin biopsy confirmed the diagnosis of EV. Immunological evaluation revealed low absolute counts of CD3 T cells, CD3+CD4, CD3+CD8, CD19, and CD16+CD56, with persistent lymphopenia (390-940 cells/mm³). Hypogammaglobulinemia was ruled out. The diagnosis of EV secondary to LOCID was confirmed, and the patient is currently on a monthly immunoglobulin transfusion program with favorable clinical outcomes. LOCID, a subset of Variable Common Immune Deficiency (VCID), is characterized by defects in antibody production and profound CD4 T-cell lymphopenia, distinguishing it from Common Variable Immune Deficiency (CVID). Systematic T-cell phenotyping may enhance diagnostic accuracy and therapeutic strategies, providing critical insights for genetic diagnosis and management of associated infections.