We initially considered a diagnosis of incontinentia pigmenti (IP), a rare X-linked dominant neuroectodermal disorder primarily caused by mutations in the IKBKG/NEMO gene, which may be associated with immunodeficiency, autoinflammatory and autoimmune diseases (e.g., inflammatory bowel disease, Behcet syndrome, systemic lupus erythematosus, etc.)(19–22). The gene discussed is IKBKG; the disease is Immunodeficiency.