Recurrent Meningitis in Hyper IgE Syndrome

Excerpt

To the Editor: A 12-y-old boy presented with altered sensorium with convulsions of 1 d. Child had been treated for chronic suppurative otitis media (CSOM) and streptococcal meningitis in preceding 2 y. Vital signs were stable with evident meningeal irritation. Patient had coarse skin with hypo pigmented macules, broad forehead, widely placed deep eyes and high-arched palate (Fig. 1). Cerebrospinal fluid analysis revealed 486 cells/mm³ with neutrophil predominance, reduced sugar and elevated protein. Gram staining and culture were negative. Ear discharge yielded Staphylococcus aureus. Computed tomography revealed bilateral mastoiditis with CSOM. IgG was 1,720 mg/dl (400–1,590 mg/dl), IgM-116 mg/dl (30–265 mg/dl), IgA-482 mg/dl (50–462 mg/dl), IgE 6,958 IU/ml (normal < 250 IU/ml), C4–51 mg/dl (14–44 mg/dl) and C3–269 mg/dl (70–170 mg/dl). Child recovered with appropriate antibiotics. Child was later immunized with H.influenza and pneumococcal polysaccharide vaccines.

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