Case report of CRIA syndrome: progression from initial response to anakinra to sustained control with tocilizumab.

We present the case of a 40-day-old female infant with acute febrile illness, cutaneous lesions, severe respiratory distress, and shock refractory to antibiotic therapy. Skin biopsy revealed findings consistent with acute febrile neutrophilic dermatosis (Sweet syndrome). After excluding infectious, hematological, and hemophagocytic etiologies, an autoinflammatory syndrome was suspected, and treatment with anakinra and glucocorticoids was initiated, resulting in initial clinical improvement. However, after one year of treatment and dose tapering, the patient experienced relapses. Tocilizumab was then started, leading to a sustained clinical response to date.