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Review
. 2022 Jan-Feb;97(1):63-74.
doi: 10.1016/j.abd.2021.05.011. Epub 2021 Nov 24.

Generalized pustular psoriasis (von Zumbusch)

Ricardo Romiti  1 , André Luís da Silva Hirayama  2 , Marcelo Arnone  1 , Renata Ferreira Magalhães  3
Affiliations
Review

Generalized pustular psoriasis (von Zumbusch)

Ricardo Romiti et al. An Bras Dermatol. 2022 Jan-Feb.

Abstract

Generalized pustular psoriasis (von Zumbusch) is a rare and acute eruption characterized by multiple sterile pustules over an erythematous and edematous background, eventually associated with psoriasis vulgaris. Classically, it manifests as a potentially severe systemic picture and demands prompt diagnosis and intervention. The duration of each flare-up and intervals between the pustular episodes is extremely variable. Recently, genetic abnormalities have been identified mainly in the familial and early variants of this disease. The therapeutic arsenal is limited; however, new drugs being evaluated aim to control both pustular flare-ups and disease recurrences.

Keywords: Acrodermatitis; Psoriasis; Psoriasis/epidemiology; Psoriasis/genetics; Psoriasis/physiopathology; Psoriasis/therapy.

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Figures

Figure 1
Figure 1
Pathogenesis of pustular psoriasis and activation of the IL-36 pathway.
Figure 2
Figure 2
IL-36 action on its receptor and activation of intracellular pathways for the activation of transcription of pro-inflammatory genes.
Figure 3
Figure 3
Early presentation of generalized pustular psoriasis.
Figure 4
Figure 4
Lake of pus.
Figure 5
Figure 5
Eruptive presentation of generalized pustular psoriasis.
Figure 6
Figure 6
Generalized presentation affecting the lower limbs.
Figure 7
Figure 7
Severe condition affecting the upper limbs.
Figure 8
Figure 8
Pustular eruption over plaques of psoriasis vulgaris.
Figure 9
Figure 9
Residual lesions of pustular psoriasis.
Figure 10
Figure 10
Pustular psoriasis: subcorneal pustule, of the spongiform type (inset, arrow). Hematoxylin & eosin, ×100 and ×400 (inset).
See this image and copyright information in PMC

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