Incidence of clinical and laboratory findings in patients with typical sporadic or autosomal dominant HIES.
Immune System
Findings | Incidence |
---|---|
Eczema | 100% |
Skin abscesses | 87% |
Recurrent pneumonia (3 or more, proven by X-ray) |
87% |
Pneumatoceles | 77% |
Mucocutaneous candidiasis | 83% |
Skeletal Symptoms
Findings | Incidence |
---|---|
Characteristic face | 83% |
Wide nose (interalar distance >2 SD above norm) |
65% |
Failure of dental exfoliation (>3 teeth) |
72% |
Hyperextensibility | 68% |
Recurrent pathologic fractures | 57% |
Scoliosis (>10°) | 63% |
Laboratory Findings
Findings | Incidence |
---|---|
Serum-IgE (>2000 U/ml or >10 times the age-specific upper norm) |
97% |
Eosinophilia (>2 SD above norm) |
93% |
Adapted from Grimbacher B, Holland SM, and Puck JM. Hyper-IgE syndromes. Immunological Reviews 203 (1), 244 – 50.