Unusual psoriasiform lesions in a patient with Hyper‐IgE syndrome

Xl, Li; Geng, S.; Xb, Lei; Xiao, Shifu; Liu, Y; Peng, Zhenhui

doi:10.1111/j.1468-3083.2006.01918.x

Cited by 4 publications

(1 citation statement)

References 5 publications

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“…Serum IgE concentrations were elevated in 81.3% of the PE group, compared to 6.3% of the control group. In addition, case studies involving psoriasiform lesions and elevated IgE concentrations have been similarly documented 47, 48 . It would thus be interesting to study the IgE concentrationss in patients carrying the Act1 (D10N) variant to determine whether this mutation defines a specific cohort of patients with psoriasis.…”

Section: Discussionmentioning

confidence: 97%

The psoriasis-associated D10N variant of the adaptor Act1 with impaired regulation by the molecular chaperone hsp90

Wang

Bulek

et al. 2012

Nat Immunol

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Act1 is an essential adaptor molecule in IL-17-mediated signaling and is recruited to the IL-17 receptor upon IL-17 stimulation. Here, we report that Act1 is a client protein of the molecular chaperone, Hsp90. The Act1 variant (D10N) linked to psoriasis susceptibility is defective in its interaction with Hsp90, resulting in a global loss of Act1 function. Act1-/- mice modeled the mechanistic link between Act1 loss of function and psoriasis susceptibility. Although Act1 is necessary for IL-17-mediated inflammation, Act1-/- mice exhibited a hyper TH17 response and developed spontaneous IL-22-dependent skin inflammation. In the absence of IL-17-signaling, IL-22 is the main contributor to skin inflammation, providing a molecular mechanism for the association of Act1 (D10N) with psoriasis susceptibility.

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Section: Discussionmentioning

confidence: 97%