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.
Keywords: Deficiency of IL-1 receptor antagonist (DIRA); PAPA syndrome; Palmoplantar pustulosis; Pustular psoriasis; SAPHO syndrome; Subcorneal pustular dermatosis.
Published by Elsevier Inc.