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Updated: May 10, 2026

Granulocyte-dependent Autoantibody-induced Skin Blistering
Published on: October 12, 2012
Autoinflammatory pustular neutrophilic diseases
1Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Building 10, Room 12N238, 10 Center Drive, MSC 1908, Bethesda, MD 20892-1908, USA.
Abstract:
This article provides a new categorization of inflammatory pustular dermatoses in the context of recent genetic and biological insights. Monogenic diseases with pustular phenotypes are discussed, including deficiency of interleukin 1 receptor antagonist, deficiency of the interleukin 36 receptor antagonist, CARD14-associated pustular psoriasis, and pyogenic arthritis, pyoderma gangrenosum, and acne. How these new genetic advancements may inform how previously described pustular diseases are viewed, including pustular psoriasis and its clinical variants, with a focus on historical classification by clinical phenotype, is also discussed.
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