Pharmacological Management of Pediatric Pustular Psoriasis

Huang, Yi-Wei; Tsai, Tsen‐Fang

doi:10.1007/s40272-020-00383-6

Cited by 8 publications

(4 citation statements)

References 153 publications

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“…Among the conventional systemic drugs, oral retinoid treatment is the most commonly administered, even if there are concerns about growth disturbances. Cyclosporine and methotrexate have also been used as first-line treatment, whereas etanercept may be regarded as one of the preferred second-line choices for children with GPP [ 167 ].…”

Section: Treatmentmentioning

confidence: 99%

Pustular Psoriasis: From Pathophysiology to Treatment

Genovese

Moltrasio

Cassano³

et al. 2021

Biomedicines

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Pustular psoriasis (PP) is a clinicopathological entity encompassing different variants, i.e., acute generalized PP (GPP), PP of pregnancy (impetigo herpetiformis), annular (and circinate) PP, infantile/juvenile PP, palmoplantar PP/palmoplantar pustulosis, and acrodermatitis continua of Hallopeau (ACH), which have in common an eruption of superficial sterile pustules on an erythematous base. Unlike psoriasis vulgaris, in which a key role is played by the adaptive immune system and interleukin (IL)-17/IL-23 axis, PP seems to be characterized by an intense inflammatory response resulting from innate immunity hyperactivation, with prominent involvement of the IL-36 axis. Some nosological aspects of PP are still controversial and debated. Moreover, owing to the rarity and heterogeneity of PP forms, data on prognosis and therapeutic management are limited. Recent progresses in the identification of genetic mutations and immunological mechanisms have promoted a better understanding of PP pathogenesis and might have important consequences on diagnostic refinement and treatment. In this narrative review, current findings in the pathogenesis, classification, clinical features, and therapeutic management of PP are briefly discussed.

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Section: Treatmentmentioning

confidence: 99%

Pustular Psoriasis: From Pathophysiology to Treatment

Genovese

Moltrasio

Cassano³

et al. 2021

Biomedicines

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“…Etanercept, a biologic TNF‐α inhibitor, is another first‐line systematic therapeutic agent for juvenile GPP 7,8,25 . Regarding the pathogenesis of GPP, TNF‐α is one of the pro‐inflammatory cytokines that induces IL‐36 expression, which further increases the production of chemokine motif ligand 1 (CXCL1), CXCL2, CXCL8 and the recruitment of neutrophils 5 .…”

Section: Discussionmentioning

confidence: 99%

“…Etanercept, a biologic TNFα inhibitor, is another first-line systematic therapeutic agent for juvenile GPP. 7,8,25 Regarding the pathogenesis of GPP, TNFα is one of the pro-inflammatory cytokines that induces IL-36 expression, which further increases the production of chemokine motif ligand 1 (CXCL1), CXCL2, CXCL8 and the recruitment of neutrophils. 5 Accordingly, the majority of genes associated with neutrophil activation, neutrophil-mediated immunity and neutrophil degranulation (cluster 1 and 2) were found downregulated in the specimen after etanercept treatment via RNAseq (Figure 2B, C), which correlated with the clinical observation of nearly complete remitted erythema and symptoms (Figure 1A).…”

Section: Clinical and Transcriptomic Response To Etanerceptmentioning

confidence: 99%

Transcriptomic responses of peripheral blood mononuclear cells to cyclosporin and etanercept in a female infant with juvenile generalized pustular psoriasis

Lin

Jeng

Aala

et al. 2023

Experimental Dermatology

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Generalized pustular psoriasis (GPP) is a rare but severe form of psoriasis. An early onset of the diseases is correlated with mutations among IL36RN, CARD14, AP1S3, MPO and SERPINA3 genes. Systemic biological agents including anti‐TNF‐α, anti‐IL‐17, anti‐IL‐12/IL‐23, anti‐IL1R, anti‐IL1β and anti‐IL‐36R act as novel treatment methods for GPP. Herein we report a female infant clinically diagnosed with GPP since she was 10‐month‐old. Results of whole‐exome sequencing (WES) and Sanger sequencing revealed a reported heterozygous IL36RN (c.115+6T>C) and another reported heterozygous SERPINA3 frame‐shifting variant (c.1247_1248del). Initial cyclosporin treatment for the patient led to a partial remission of the symptoms. However, the patient reached nearly total remission of pustules and erythema after anti‐TNF‐α inhibitor etanercept treatment. Results of further RNA sequencing (RNA‐seq) done on peripheral blood mononuclear cells correlated with the clinical responses, showing that cyclosporin suppressed a portion of the neutrophil‐related genes, while most genes associated with neutrophil activation, neutrophil‐mediated immunity and degranulation were downregulated by the subsequent etanercept treatment. We report this case to demonstrate WES and RNA‐seq in combination could come in handy in reaching a precise diagnosis and in evaluating or even predicting the molecular alterations underlying clinical treatment effectiveness.

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“…В настоящее время таргетного лечения ГПП не существует, а спектр патогенетических методов лечения, позволяющих добиться длительной ремиссии, ограничен [11]. В частности, для лечения ГПП рекомендовано назначение ретиноидов, системных пероральных или парентеральных глюкокортикостероидов, циклоспорина или метотрексата [14,15]. Указанные препараты эффективны у 70-84% пациентов [16].…”

Section: клиническое наблюдениеunclassified

Infiltrative-Suppurative Form of Scalp Microsporia in a Child with Generalized Pustular Psoriasis of von Zumbusch: Clinical Case

Бакулев

Kravchenya

Igonina

2021

Vopr. sovr. pediatr.

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Background. This article discusses issues of diagnosis and management of pustular psoriasis in a child associated with recurrent dermatomycosis, immunopathogenetic mechanisms of pustular psoriasis, its course and prognosis. Clinical Case Description. The clinical case of infiltrative-suppurative form of scalp microsporia in 11 years old child with long-term psoriasis vulgaris and its transformation to generalized pustular psoriasis (von Zumbusch) is described. The need of immunosuppressive therapy prescription for severe psoriasis was challenged with fungal skin infection as immune suppression would lead to its recurrent course. Conclusion. Combination treatment (systemic glucocorticosteroids, methotrexate, antibiotics, and griseofulvin) resulted in clinical and laboratory recovery for microsporia in a child with generalized pustular psoriasis.

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Pharmacological Management of Pediatric Pustular Psoriasis

Cited by 8 publications

References 153 publications

Pustular Psoriasis: From Pathophysiology to Treatment

Pustular Psoriasis: From Pathophysiology to Treatment

Transcriptomic responses of peripheral blood mononuclear cells to cyclosporin and etanercept in a female infant with juvenile generalized pustular psoriasis

Infiltrative-Suppurative Form of Scalp Microsporia in a Child with Generalized Pustular Psoriasis of von Zumbusch: Clinical Case

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