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.