From: The hyperimmunoglobulin E syndrome - clinical manifestation diversity in primary immune deficiency
CLINICAL FINDINGS | points | |||||||||
---|---|---|---|---|---|---|---|---|---|---|
 | 0 | 1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 10 |
Highest serum-IgE level (IU/ml) [Normal < 130 IU/ml] | < 200 | 200-500 | Â | Â | 501-1000 | Â | Â | Â | 1001-2000 | > 2000 |
Skin abscesses | None | Â | 1-2 | Â | 3-4 | Â | Â | Â | > 4 | Â |
Pneumonia (episodes over lifetime) | None | Â | 1 | Â | 2 | Â | 3 | Â | > 3 | Â |
Parenchymal lung anomalies | Absent | Â | Â | Â | Â | Â | Bronchiectasis | Â | Pneumatocele | Â |
Retained primary teeth | None | 1 | 2 | Â | 3 | Â | Â | Â | > 3 | Â |
Scoliosis, maximum curvature | < 100 | Â | 10-140 | Â | 15-200 | Â | Â | Â | > 200 | Â |
Fractures with minor trauma | None | Â | Â | Â | 1-2 | Â | Â | Â | > 2 | Â |
Highest eosinophil count (cells/μl) [700/μl = 1 SD, 800/μl = 2 SD] | < 700 |  |  | 700-800 |  |  | > 800 |  |  |  |
Characteristic face | Absent | Â | Mildly present | Â | Â | Present | Â | Â | Â | Â |
Midline anomaly (cleft palate, cleft tongue, hemivertebrae, other vertebral anomaly) | Absent | Â | Â | Â | Â | Present | Â | Â | Â | Â |
Newborn rash | Absent | Â | Â | Â | Present | Â | Â | Â | Â | Â |
Eczema (worst stage) | Absent | Mild | Moderate | Â | Severe | Â | Â | Â | Â | Â |
Upper respiratory infections per year | 1-2 | 3 | 4-6 | Â | > 6 | Â | Â | Â | Â | Â |
Candidiasis | None | Oral | Fingernails | Â | Systemic | Â | Â | Â | Â | Â |
Other serious infections | None | Â | Â | Â | Severe | Â | Â | Â | Â | Â |
Fatal infection | Absent | Â | Â | Â | Present | Â | Â | Â | Â | Â |
Hyperextensibility | Absent | Â | Â | Â | Present | Â | Â | Â | Â | Â |
Lymphoma | Absent | Â | Â | Â | Present | Â | Â | Â | Â | Â |
Increased nasal width | < 1 SD | 1-2 SD | Â | > 2 SD | Â | Â | Â | Â | Â | Â |
High palate | Absent | Â | Present | Â | Â | Â | Â | Â | Â | Â |
Young-age correction | > 5 years |  |  | 2-5 years |  | 1-2 years |  | ≤ 1 year |  |  |