Familial juvenile generalized pustular psoriasis

Hubler, W. R.

doi:10.1001/archderm.120.9.1174

Cited by 9 publications

(6 citation statements)

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“…A personal or family history of psoriasis may be present in these patients 91, 119, 120 . Disease onset is typically in the third trimester, although it may occur earlier in pregnancy, and both cutaneous and systemic symptoms resolve gradually following delivery or termination of pregnancy 15, 91, 120–122 .…”

Section: Pustular Psoriasis and Clinical Variantsmentioning

confidence: 99%

Autoinflammatory Pustular Neutrophilic Diseases

Naik

Cowen

2013

Dermatologic Clinics

114

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SYNOPSIS The inflammatory pustular dermatoses constitute a spectrum of non-infectious conditions ranging from localized involvement to generalized disease with associated acute systemic inflammation and multi-organ involvement. Despite the variability in extent and severity of cutaneous presentation, each of these diseases is characterized by non-infectious neutrophilic intra-epidermal microabscesses. Many share systemic findings including fever, elevated inflammatory markers, inflammatory bowel disease and/or osteoarticular involvement, suggesting potential common pathogenic links (Figure 1). The recent discoveries of several genes responsible for heritable pustular diseases have revealed a distinct link between pustular skin disease and regulation of innate immunity. These genetic advances have led to a deeper exploration of common pathways in pustular skin disease and offer the potential for a new era of biologic therapy which targets these shared pathways. This chapter provides a new categorization of inflammatory pustular dermatoses in the context of recent genetic and biologic insights. We will discuss recently-described monogenic diseases with pustular phenotypes, including deficiency of IL-1 receptor antagonist (DIRA), deficiency of the IL-36 receptor antagonist (DITRA), CARD14-associated pustular psoriasis (CAMPS), and pyogenic arthritis, pyoderma gangrenosum, acne (PAPA). We will then discuss how these new genetic advancements may inform how we view previously described pustular diseases, including pustular psoriasis and its clinical variants, with a focus on historical classification by clinical phenotype.

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Section: Pustular Psoriasis and Clinical Variantsmentioning

confidence: 99%

Autoinflammatory Pustular Neutrophilic Diseases

Naik

Cowen

2013

Dermatologic Clinics

114

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“…It is characterized by waves of widespread sheets of sterile pustules on brightly erythematous skin, high fever and general malaise. The occurrence of GPP in childhood is rare, and fewer than 200 cases have been reported in the published work 2–7 . We herein report an 8‐year‐old Chinese boy with juvenile GPP and review 12 juvenile GPP inpatients treated at our hospital in the period 1978–2005.…”

Section: Introductionmentioning

confidence: 97%

Juvenile generalized pustular psoriasis

Xiao

et al. 2007

The Journal of Dermatology

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“… 75 The disease has been reported in several sibling or other familial associations, lending to the theory of at least a partial genetic component to the dermatosis. 78 Juvenile pustular psoriasis, like other forms of psoriasis, is often associated with identifiable triggers such as infection, vaccination, and corticosteroid withdrawal. 79 …”

Section: Generalized Pustular Psoriasismentioning

confidence: 99%

Pustular psoriasis: pathophysiology and current treatment perspectives

Benjegerdes¹,

Hyde²,

Kivelevitch³

et al. 2016

PTT

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Psoriasis vulgaris is a chronic inflammatory disease that classically affects skin and joints and is associated with numerous comorbidities. There are several clinical subtypes of psoriasis including the uncommon pustular variants, which are subdivided into generalized and localized forms. Generalized forms of pustular psoriasis include acute generalized pustular psoriasis, pustular psoriasis of pregnancy, and infantile and juvenile pustular psoriasis. Localized forms include acrodermatitis continua of Hallopeau and palmoplantar pustular psoriasis. These subtypes vary in their presentations, but all have similar histopathologic characteristics. The immunopathogenesis of each entity remains to be fully elucidated and some debate exists as to whether these inflammatory pustular dermatoses should be classified as entities distinct from psoriasis vulgaris. Due to the rarity of these conditions and the questionable link to the common, plaque-type psoriasis, numerous therapies have shown variable results and most entities remain difficult to treat. With increasing knowledge of the pathogenesis of these variants of pustular psoriasis, the development and use of biologic and other immunomodulatory therapies holds promise for the future of successfully treating pustular variants of psoriasis.

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Familial juvenile generalized pustular psoriasis

Cited by 9 publications

References 0 publications

Autoinflammatory Pustular Neutrophilic Diseases

Autoinflammatory Pustular Neutrophilic Diseases

Juvenile generalized pustular psoriasis

Pustular psoriasis: pathophysiology and current treatment perspectives

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