Pustular psoriasis and related pustular skin diseases

@article{Bachelez2018PustularPA,
  title={Pustular psoriasis and related pustular skin diseases},
  author={Herv{\'e} Bachelez},
  journal={British Journal of Dermatology},
  year={2018},
  volume={178}
}
  • H. Bachelez
  • Published 1 March 2018
  • Medicine
  • British Journal of Dermatology
Patients with pustular psoriasis or related pustular diseases may have genetic abnormalities impairing the function of key players of the innate skin immune system. Recently, identification of these abnormalities has changed the paradigm of several of these diseases. These include generalized pustular psoriasis, palmoplantar pustular psoriasis and acrodermatitis continua of Hallopeau, and also drug‐induced acute exanthematous generalized pustular eruption. Identified mutations in IL36RN, CARD14… 
Generalized Pustular Psoriasis: Divergence of Innate and Adaptive Immunity
TLDR
The novel anti-IL-36R antibodies are particularly promising and may revolutionize management of the disease.
Pustular Psoriasis: A Narrative Review of Recent Developments in Pathophysiology and Therapeutic Options
TLDR
The clinical features of PPP and GPP are reviewed, and current understanding of the genetics and immunopathology of these conditions are reviewed; it also provides an update on emerging treatments.
Generalized Pustular Psoriasis: Clinical Management and Update on Autoinflammatory Aspects
TLDR
Challenges regarding the precise clinical diagnosis and evaluation of clinical symptoms in GPP are addressed, and the current understanding of molecular pathomechanisms behind GPP as an autoinflammatory keratinization disease is updated and summarized.
Pustular Psoriasis: From Pathophysiology to Treatment
TLDR
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.
Autoinflammatory diseases predominantly affecting the skin
TLDR
Several syndromes with skin findings ranging from cold-induced urticaria, neutrophilic granulomas, sterile cellulitis, and pustular psoriasis are focused on, establishing the skin as truly the window to diagnosis of these immunodysregulatory disorders.
Variants of CARD14 are predisposing factors for generalized pustular psoriasis (GPP) with psoriasis vulgaris but not for GPP alone in a Chinese population
  • L Li, J. You, +4 authors F. Zhang
  • Medicine
    The British journal of dermatology
  • 2019
TLDR
GPP is traditionally classified as the most severe form of psoriasis, although some scholars have proposed that GPP alone can be treated as a distinct etiology because that this condition is strikingly different from PV in terms of its clinical, histological and genetic background.
Biologics in the treatment of pustular psoriasis
TLDR
This review focuses on the efficacy and safety of biologics, including anti-IL‐1β (gevokizumab and canakinumab), anti- IL‐1 R (anakinra), pro-IL-36 R (BI 655130), and anti-tumor necrosis factor-α (etanercept, infliximab, and adalimumab), for treating pustular psoriasis.
Treatment and Management of Psoriasis: A review
Psoriasis is a common, chronic inflammatory skin disease affecting many people of the world now a days. Psoriasis is principally an immunological T lymphocyte-driven disease, relating both the
Successful therapy with secukinumab in a patient with generalized pustular psoriasis carrying homozygous IL36RN p.His32Arg mutation
TLDR
The efficiency of secukinumab, an IL-17 inhibitor, is described in a patient with GPP and carrying a homozygous IL36RN p.His32Arg mutation, previously described as a hypomorphic mutation with reduced IL-36Ra protein expression.
Histologic Patterns and Clues to Autoinflammatory Diseases in Children: What a Cutaneous Biopsy Can Tell Us
TLDR
It is important for a dermatopathologist to recognize the patterns of autoinflammation, so that a diagnosis of MAIS or complex multigenic diseases may be obtained, and careful histopathologic analyses could contribute to a better understanding of the various clinical manifestations of auto inflammation.
...
1
2
3
4
...

References

SHOWING 1-10 OF 45 REFERENCES
Activating CARD14 Mutations Are Associated with Generalized Pustular Psoriasis but Rarely Account for Familial Recurrence in Psoriasis Vulgaris.
TLDR
A systematic CARD14 analysis in an extended patient cohort indicated that the analysis of CARD14 mutations could help stratify pustular psoriasis cohorts but would be mostly uninformative in the context of Psoriasis and sporadic PRP.
Pustular and erythrodermic psoriasis complicated by acute respiratory distress syndrome.
TLDR
Generalized pustular and erythrodermic psoriasis may be complicated by ARDS, the pathogenesis of which is unclear, but proinflammatory cytokines may be involved.
Generalized pustular psoriasis. A review of 63 cases.
TLDR
Four subgroups of patients with generalized pustular psoriasis were classified on the basis of onset and morphologic pattern of disease: acute (von Zumbusch), subacute annular, chronic (acral), and mixed to provide a better understanding of the variability of the disease.
Pustular psoriasis and aseptic purulent arthritis: possible role of leukotrienes B4 and C4 in a flare of synovitis.
TLDR
i-LTB4 and i-LTC4 appear to be generated in the arthritis lesions of pustular psoriasis, the former attracting PMNs to the joints and the latter causing exudation of synovial fluid.
Correlation of IL36RN mutation with different clinical features of pustular psoriasis in Chinese patients
TLDR
The c.115+6T>C mutation was the most common and the most important variant in all subtypes of pustular psoriasis with IL36RN mutations among the authors' sample of Chinese patients.
THE PROGNOSIS OF GENERALIZED PUSTULAR PSORIASIS TERENCE
TLDR
Data concerning 155 patients with all types of generalized pustular psoriasis have been reviewed and it is shown that developing from acral pustulosis (acrodermatitis continua) has the worst prognosis and is likely to prove fatal.
The majority of generalized pustular psoriasis without psoriasis vulgaris is caused by deficiency of interleukin-36 receptor antagonist.
TLDR
GPP alone is a distinct subtype of GPP and is etiologically distinguished from GPP with PV, and that the majority of G PP alone is caused by deficiency of the interleukin-36 receptor antagonist due to IL36RN mutations.
Therapeutic guidelines for the treatment of generalized pustular psoriasis (GPP) based on a proposed classification of disease severity
TLDR
Following the development of a unique classification of the disease severity based on scoring the clinical symptoms and the laboratory findings, therapeutic guidelines for the treatment of GPP are proposed.
Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis.
TLDR
Aberrant interleukin-36Ra structure and function lead to unregulated secretion of inflammatory cytokines and generalized pustular psoriasis.
Mutation Analysis of the IL36RN Gene in 14 Japanese Patients with Generalized Pustular Psoriasis
TLDR
Mutation analysis of the IL36RN gene was performed in 14 Japanese patients with GPP and identified mutations in two patients analyzed, which further underscore the critical role of IL 36RN in pathogenesis of GPP.
...
1
2
3
4
5
...