Clinical Features
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, Bodo, Holland, Steven M. & Puck, Jennifer M. Hyper-IgE syndromes. Immunological Reviews 203 (1), 244 – 50.
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