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Review
. 2013 Jul;31(3):405-25.
doi: 10.1016/j.det.2013.04.001. Epub 2013 Jun 2.

Autoinflammatory pustular neutrophilic diseases

Haley B Naik  1 , Edward W Cowen
Affiliations
Review

Autoinflammatory pustular neutrophilic diseases

Haley B Naik et al. Dermatol Clin. 2013 Jul.

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.

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

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Figures

Figure 1
Figure 1. Overlapping clinical features of pustular dermatoses
CARD 14-mediated pustular psoriasis (CAMPS); deficiency of IL-1 receptor antagonist (DIRA); deficiency of the IL-36 receptor antagonist (DITRA); generalized pustular psoriasis (GPP); pyogenic arthritis, pyoderma gangrenosum, and acne (PAPA); pyoderma, acne and suppurative hidradenitis (PASH); palmoplantar pustulosis (PPP); synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO); subcorneal pustular dermatosis (SCPD).
Figure 2
Figure 2. Deficiency of IL-2 receptor antagonist (DIRA)
Bright red plaques studded with crops of pustules in an infant. Photo courtesy of Raphaela Goldbach-Mansky.
Figure 3
Figure 3. PAPA syndrome
Cribiform pyoderma gangrenosum ulcers in the setting of severe scarring from acne conglobata.
Figure 4
Figure 4. Annular pustular psoriasis
Gyrate plaques of annular pustular psoriasis notable for central sparing and erythematous border studded with pustules and yellow crusting. Photo courtesy of Jeffrey Callen.
Figure 5
Figure 5. Subcorneal pustular dermatosis
Superficial pustules and scale collarettes in annular and gyrate patterns overlying a relatively non-inflammatory background.
Figure 6
Figure 6. Palmoplantar pustular psoriasis
Pustules, scaling and painful erosions with overlying hemorrhagic crust on the palmar surfaces. Photo courtesy of Kristina Callis-Duffin.
See this image and copyright information in PMC

References

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