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Hyper-IgE syndrome

MONDO:0018037

A condition that is characterized by elevated serum IgE, dermatitis, and respiratory infections.

Also known as: HIES, hyper-IgE recurrent infection syndrome, hyperimmunoglobulin E syndrome

66 clinical trials for this condition and its sub-types, 5 tagged with Hyper-IgE syndrome itself.

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Where it sits in the disease tree

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↑ Hyperimmunoglobulin syndrome (34)

Sub-types of Hyper-IgE syndrome

Netherton syndrome 16 trials
Combined immunodeficiency due to DOCK8 deficiency 6 trials
Hyper-IgE recurrent infection syndrome 1, autosomal dominant 3 trials
Autosomal dominant combined immunodeficiency due to ERBIN deficiency 0 trials
Autosomal dominant combined immunodeficiency due to partial IL6ST deficiency 0 trials
Autosomal recessive combined immunodeficiency due to IL6R deficiency 0 trials
Autosomal recessive combined immunodeficiency due to complete IL6ST deficiency 0 trials
Autosomal recessive combined immunodeficiency due to partial IL6ST deficiency 0 trials
Hyper-IgE recurrent infection syndrome 3, autosomal recessive 0 trials
Hyper-IgE recurrent infection syndrome 4, autosomal recessive 0 trials
Hyper-IgE recurrent infection syndrome 4A, autosomal dominant 0 trials
Hyper-IgE recurrent infection syndrome 5, autosomal recessive 0 trials
Hyper-IgE syndrome 6, autosomal dominant, with recurrent infections 0 trials
Immunodeficiency 94 with autoinflammation and dysmorphic facies 0 trials

Including sub-types (66) Tagged with Hyper-IgE syndrome (5)

Trials to join now! 33 Not yet recruiting 5 Not yet finished but already full! 9 Completed 15 Terminated 4

Experimental drug for rare skin disease fails to reach goal

Disease control Stopped early

This early-stage trial tested a drug called DS-2325a in 9 adults with Netherton syndrome, a rare genetic condition causing severe skin redness, scaling, and allergies. The study aimed to check safety and whether the drug could help control the disease. However, the trial was term…

Phase 1/2 • Sponsor: Daiichi Sankyo • Aim: Disease control

Last updated Jun 27, 2026 12:33 UTC
Hope for rare skin disease: new drug shows promise in trial

Disease control Stopped early

This study tested a medicine called spesolimab for people with Netherton syndrome, a rare genetic skin condition causing severe redness and scaling. About 43 people aged 12 and older took part, receiving either the drug or a placebo. The goal was to see if spesolimab could reduce…

Phase 2/3 • Sponsor: Boehringer Ingelheim • Aim: Disease control

Last updated Jun 27, 2026 11:00 UTC
Can an eczema drug calm a rare genetic skin disease?

Symptom relief Stopped early

Netherton syndrome is a rare genetic disorder that causes red, scaly, inflamed skin and intense itching. No targeted treatments exist, so researchers are testing dupilumab, an injectable drug already used for eczema, against a placebo in 10 adults with moderate to severe Netherto…

Phase 2/3 • Sponsor: University Hospital, Toulouse • Aim: Symptom relief

Last updated Sep 22, 2026 00:00 UTC
Rare skin disease study seeks to understand netherton syndrome

Knowledge-focused Stopped early

This study aimed to collect real-world data on Netherton Syndrome, a rare genetic skin disorder. Researchers planned to follow 4 participants over 52 weeks, measuring skin severity and other symptoms. The study was terminated early, so results are limited.

Sponsor: Boehringer Ingelheim • Aim: Knowledge-focused

Last updated Jun 27, 2026 14:03 UTC

Hyper-IgE syndrome

Where it sits in the disease tree

Experimental drug for rare skin disease fails to reach goal

Hope for rare skin disease: new drug shows promise in trial

Can an eczema drug calm a rare genetic skin disease?

Rare skin disease study seeks to understand netherton syndrome