Asian Journal of Case Reports in Medicine and Health

Introduction: Chronic granulomatous disease (CGD) is a rare inherited primary immunodeficiency characterized by defective oxidative metabolism of phagocytic cells. It is occasionally associated with autoimmune manifestations, including systemic lupus erythematosus (SLE). Sweet’s syndrome, also called acute febrile neutrophilic dermatosis, is another rare condition. Its association with SLE remains uncommon.                                  

Case Presentation: We report the case of a 40-year-old Moroccan woman, born from a consanguineous marriage, with a history of recurrent bacterial infections since childhood. At the age of 32, she developed severe bacterial meningitis, and further investigations revealed CGD with markedly reduced neutrophil oxidative activity on nitroblue tetrazolium testing. In 2019, she was diagnosed with SLE according to the ACR/EULAR 2018 criteria. In 2021, she presented with painful ulcerated papulo-squamous skin lesions, severe polyarthralgia, and episcleritis. Skin biopsy demonstrated a dermal neutrophilic infiltrate consistent with Sweet’s syndrome. She was treated with systemic corticosteroids, with complete resolution.      

Conclusion: This case illustrates the exceptional coexistence of CGD, SLE, and Sweet’s syndrome in the same patient. It also highlights the therapeutic challenge of balancing immunosuppressive therapy, which is required to control autoimmune and autoinflammatory manifestations, against the heightened risk of severe infections inherent to CGD. Early recognition and a carefully individualized approach are essential to optimize outcomes.