Clinical Features of Human Hyper IgE Syndrome (STAT3base)

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.

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