Table 1 Scoring System with Clinical and Laboratory Tests for Related Individuals with HIES

Highest serum-IgE level (IU/ml)

[Normal < 130 IU/ml]

< 200

200-500

501-1000

1001-2000

> 2000

Skin abscesses

None

1-2

3-4

> 4

Pneumonia (episodes over lifetime)

None

1

2

3

> 3

Parenchymal lung anomalies

Absent

Bronchiectasis

Pneumatocele

Retained primary teeth

None

1

2

3

> 3

Scoliosis, maximum curvature

< 10⁰

10-14⁰

15-20⁰

> 20⁰

Fractures with minor trauma

None

1-2

> 2

Highest eosinophil count (cells/μl)

[700/μl = 1 SD, 800/μl = 2 SD]

< 700

700-800

> 800

Characteristic face

Absent

Mildly present

Present

Midline anomaly (cleft palate, cleft tongue, hemivertebrae, other vertebral anomaly)

Absent

Present

Newborn rash

Absent

Present

Eczema (worst stage)

Absent

Mild

Moderate

Severe

Upper respiratory infections per year

1-2

3

4-6

> 6

Candidiasis

None

Oral

Fingernails

Systemic

Other serious infections

None

Severe

Fatal infection

Absent

Present

Hyperextensibility

Absent

Present

Lymphoma

Absent

Present

Increased nasal width

< 1 SD

1-2 SD

> 2 SD

High palate

Absent

Present

Young-age correction

> 5 years

2-5 years

1-2 years

≤ 1 year