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Table 1 Distinguishing Features Comparing AD-HIES With DIDS and TYK2 HIES

From: The Hyper-IgE Syndromes: Lessons in Nature, From Bench to Bedside

Clinical Finding

AD-HIES

DIDS

AR-HIES

TYK2

AR-HIES

Viral skin infections

-

+++

++

Pneumatoceles

++

-

-

Recurrent pneumonia

++

++

++

Sepsis mortality

+

+++

+++

Facial dysmorphic features

+++

-

-

Primary tooth retention

+++

-

-

Scoliosis

++

-

-

Neurologic manifestations

+

+++

++

Cerebral vascular abnormalities

+

++

++

Food allergies

-

+++

-

Environmental allergies

-

++

 

Decreased IgM

-

++

-

Atypical mycobacterial infections

-

-

++

Malignancy

+

+++

?

Lymphoma

+

+

?