Split Immunological Reconstitution in a NEMO-Deficient Male with Incontinentia Pigmenti and Immunodeficiency.

This document is a letter published in the Journal of Clinical Immunology. It discusses a case study of a male patient with Incontinentia Pigmenti and X-linked anhidrotic ectodermal dysplasia with immunodeficiency (IP-EDA-ID). The patient had a pathogenic variant in the NEMO/IKBKG gene, which caused split immunological reconstitution. The study examines the chronological changes in somatic mosaicism of T cells, B cells, and monocytes in the patient. The findings suggest that monitoring mosaicism can guide the appropriate treatment for NEMO deficiency. [Extracted from the article]