White male, born in 2001, a full sibling of Case 2. BCG-vaccinated during the first month of life. At age 4 months, he had regional suppurative adenitis ipsilateral to the BCG vaccine site and was treated with Isoniazid (10 mg/kg/day) for 6 months with good clinical response. At age 5 years, when his older brother (Case 2) was diagnosed with histoplasmosis, he presented with multiple cervical lymphadenopathies and fever. He was treated with ketoconazole (400 mg/day). Treatment was interrupted 7 months later without recovery. At age 6 years, he was hospitalized with cervical lymphadenopathies and was treated with Amphotericin B without regression. At age 8 years, he was hospitalized with asthenia, anorexia, abdominal pain and cervical adenopathies, and evolved hepatic and splenic enlargements, respiratory distress, persistent fever, pericardial and pleural collections and splenic abscess, which demanded drainage and resection, respectively. Laboratory studies showed leukocytosis (26,900 cells/mm3; 14% bands, 80% segments, 5% lymphocytes) with normal platelet count (297,000/mm3), AST (38 U/L), ALT (25 IU/L), AP (342 IU/L), and altered albumin (2.30 g/dL), globulin (3.60 g/dL); total bilirubin (7.80 mg/dL); and direct bilirubin (6.60 mg/dL). The Wade, PAS and Giemsa stains of lymph nodes and spleen were negative. The PAS and Wade stains of the pericardial membrane were negative. The culture of bone marrow aspirate was positive for Salmonella choleraesuis. Besides antibacterial therapy (40 mg/kg/day of Vancomycin and 30 mg/kg/day of Meropenem), Rifampin (10 mg/kg/day), Isoniazid (10 mg/kg/day), and Pyrazinamide (20 mg/kg/day) were administered due to polyserositis and the unconfirmed possibility of household contacts with tuberculosis. Immunological evaluation revealed: hyper-IgE (902 and 2,848 IU/mL (RV at 87 IU/ml) in two separate evaluations as well as elevated values for IgG = 3,150 mg/dL (RV: 572-1,474), IgA = 943 mg/dL (RV: 34-305), IgM = 223 mg/dL (RV: 32-208), and T cell markers (CD4 = 955 cells/mm3 (17%), CD8 = 1,348 cells/mm3 (24%), CD4/CD8 = 0.7. Three months after suspension of the tuberculostatics, there was a recrudescence of cervical lymphadenopathy with fistulization despite the absence of fever and inflammatory signs. Cultures of ganglion aspirate were negative for bacteria, acid-fast bacilli, and fungi. Histopathology of cervical lymph nodes was negative for intracellular microbes. He received experimental treatment with Trimethoprim/Sulfamethoxazole for 5 months with complete recovery and prophylaxis with the same medication for 6 months. After the interruption of prophylaxis, he developed a left infracostal large abscess (Figure 1C), which was drained and found negative on culture. He was empirically prescribed with Ciprofloxacin (40 mg/kg/day) for Salmonella and Clarithromycin (15 mg/kg/day) for mycobacteria for 6 months. He is currently on prophylaxis with Trimethoprim/Sulfamethoxazole and is free of symptoms.