Atopic dermatitis, STAT3- and DOCK8-hyper-IgE syndromes differ in IgE-based sensitization pattern

Boos, Annette; Hagl, Beate; Schlesinger, A.; Halm, B. E.; Ballenberger, Nikolaus; Pinarci, M.; Heinz, Valerie; Kreilinger, D.; Spielberger, Benedikt D.; Schimke-Marques, L. F.; Sawalle-Belohradsky, Julie; Belohradsky, Bernd H.; Przybilla, B.; Schaub, Bianca; Wollenberg, Andreas; Renner, Ellen D.

doi:10.1111/all.12416.

Cited by 16 publications

(32 citation statements)

References 44 publications

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“…On the one hand, Boos et al had described higher frequencies of Th2 cells in patients with AD compared to healthy controls. On the other hand, they described comparable Th2 responses of patients with HIES and healthy controls . Furthermore, these data are in line with previous reports describing normal frequencies of CD4+ IL‐4+ cells, but elevated CD4+ IL‐13+ cells , and normal Th2 responses in STAT3‐HIES patients.…”

Section: Discussionsupporting

confidence: 88%

“…On the other hand, they described comparable Th2 responses of patients with HIES and healthy controls . Furthermore, these data are in line with previous reports describing normal frequencies of CD4+ IL‐4+ cells, but elevated CD4+ IL‐13+ cells , and normal Th2 responses in STAT3‐HIES patients. Collectively, these data suggest that a disturbed immune balance causes the atopic pathology rather than a strictly isolated increased Th2 response.…”

Section: Discussionsupporting

confidence: 88%

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Th2 and Th9 responses in patients with chronic mucocutaneous candidiasis and hyper‐IgE syndrome

Becker

Rösler

Wang

et al. 2016

Clin Experimental Allergy

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Section: Discussionsupporting

confidence: 88%

Section: Discussionsupporting

confidence: 88%

Th2 and Th9 responses in patients with chronic mucocutaneous candidiasis and hyper‐IgE syndrome

Becker

Rösler

Wang

et al. 2016

Clin Experimental Allergy

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“…Increased IgE levels up to 2000 U/ml are common in patients with AD-HIES, often higher than 5000 U/ml. Despite the high levels of IgE, the patients with AD-HIES suffer from a deficiency of allergic sensitization [27]; this can be explained by a normal skin barrier function and confirm its significance in the development of allergic sensitization.…”

Section: Hyper-ige Syndrome (Job's Syndrome)mentioning

confidence: 70%

“…The severity of the disease can vary. AD-HIES is associated with heterozygous mutations of signal transducer and activator of transcription three genes (STAT3; 17q21.31) [27].…”

Section: Hyper-ige Syndrome (Job's Syndrome)mentioning

confidence: 99%

Allergic Manifestations of Primary Immunodeficiency Diseases and Its Treatment Approaches

Boyarchuk

2018

Asian J Pharm Clin Res

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Allergic manifestations are one of the clinical signs of primary immunodeficiency diseases (PID). In this review, the common allergic manifestations of PID are summarized, and their main differential characteristics and treatment approaches are outlined. Allergic manifestations occur more often in patients with combined immunodeficiencies with or without associated or syndromic features. In patients with PID they usually are present in the 1st year of life, may be among the first symptoms of PID, and are commonly manifested by eczema and increased immunoglobulin (Ig)-G levels. Often the skin barrier function is not impaired in patients with eczema and PID, although some diseases (such as Comel-Netherton syndrome) do affect skin barrier function. There is usually no correlation between IgE levels and the severity of allergic skin manifestations. Allergic-like manifestations in PID patients include urticaria-like rash and angioedema. Urticaria-like rash is associated with autoinflammatory disorders, which are commonly accompanied by fever, and caused by a neutrophilic infiltrate in the dermis. Angioedema in hereditary angioedema patients is caused by high bradykinin production. Early differentiation of allergic manifestations in PID from atopic dermatitis and other atopic conditions is very difficult; however, it is very important because it influences on treatment methods. A multidisciplinary approach to the management of PID patients, with the involvement of immunologists, allergists, and formulation of appropriate treatment improve the prognosis and quality of life of the PID patients.

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“…1 AD HIES also affects T-helper 17 cells, which are critical for protection against extracellular bacteria and fungi, increasing susceptibility to Staphylococcus and Candida species infections. 2 Both AD HIES and autosomal-recessive HIES (including DOCK8 deficiency and phosphoglucomutase 3 mutations) are considered combined immunodeficiencies, although the clinical manifestations differ. 3 Patients with DOCK8 deficiency have recurrent, severe cutaneous viral infections including HSV, human papillomavirus, and molluscum contagiosum infections, and have high rates of malignancy and mortality, emphasizing the importance of early referral for HSCT.…”

Section: Pathogenesis and Clinical Presentationmentioning

confidence: 99%

Early Diagnosis in Dedicator of Cytokinesis 8 (DOCK8) Deficiency

Chang

Toh

Vos

et al. 2016

The Journal of Pediatrics

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Atopic dermatitis, STAT3- and DOCK8-hyper-IgE syndromes differ in IgE-based sensitization pattern

Cited by 16 publications

References 44 publications

Th2 and Th9 responses in patients with chronic mucocutaneous candidiasis and hyper‐IgE syndrome

Th2 and Th9 responses in patients with chronic mucocutaneous candidiasis and hyper‐IgE syndrome

Allergic Manifestations of Primary Immunodeficiency Diseases and Its Treatment Approaches

Early Diagnosis in Dedicator of Cytokinesis 8 (DOCK8) Deficiency

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