Acneiform Granulomatous Folliculitis in a Patient with Interferon-γ Deficiency: Clearing with Empiric Antimycobacterial Therapy

A young adult patient with dysmorphic facial features, cognitive impairment, congenital sensorineural hearing loss, verrucca vulgaris, and fungal nail infections presented with a worsening acneiform granulomatous folliculitis that failed to respond to aggressive medical management. An extensive evaluation was negative for specific evidence of infection, but did demonstrate an immune defect characterized by anergy and defective interferon-γ (IFN-γ) and tumor necrosis factor-α (TNF-α) synthesis. Empiric treatment for presumed atypical mycobacteria resulted in a dramatic resolution of the granulomatous folliculitis, and he has remained clear on IFN-γ therapy.