Autoinflammatory pustular neutrophilic diseases.

Dermatol Clin 2013 Jul 2;31(3):405-25. Epub 2013 Jun 2.

Dermatology 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.

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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http://dx.doi.org/10.1016/j.det.2013.04.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3703099PMC
July 2013
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