Hyperimmunoglobulin E syndrome in adult patients: a case report and literature review

The clinical manifestations, laboratory test results, treatments and prognoses of 5 adult patients with hyperimmunoglobulin E syndrome (HIES) were retrospectively analysed. All 5 patients experienced disease onset within 1 year of birth and had recurrent multiple infections, all had recurrent rash and 4 had skeletal abnormalities. Recurrent infections lasted for a mean of 19.6 years and predominantly affected the skin and respiratory tract. Other manifestations included chronic otitis media, renal abscess and osteomyelitis. Two cases had skin abscess (methicillin-susceptible Staphylococcus aureus infection), which was alleviated after antibiotic treatment and abscess drainage. Two cases had respiratory tract infection and one case had a foot skin ulcer, and all symptoms improved after antibiotic treatment. HIES should be suspected when an adult patient experiences repeated infection since birth, particularly when skeletal abnormalities and a history of neonatal rash is also present. Early diagnosis facilitates targeted anti-infection therapy, leading to earlier remission and an improved prognosis. Key words: Hyperimmunoglobulin E; Infection