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Generalized pustular psoriasis
Generalized pustular psoriasis (GPP) is a rare, severe form of pustular psoriasis characterized by recurrent flares of widespread sterile pustules with erythematous, painful skin
Prevalence
1-9 / 1 000 000
Age of Onset
ICD-10
L40.1
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
5 Facts you should know
FACT
Key symptoms of GPP include sterile pustules, pain, scaling, erythema, dryness, itching, burning, joint pain, leukocytosis, headache, fever, and fatigue
FACT
Generalized pustular psoriasis (GPP) is heterogenous and can be a relapsing disease with recurrent flares or a persistent disease with intermittent flares
FACT
GPP is phenotypically, histopathologically, and genetically distinct from plaque psoriasis
FACT
GPP is considered a neutrophilic disease, whereas plaque psoriasis is considered an autoimmune disease
FACT
The IL-36 pathway plays a key role in the pathogenesis of GPP, leading to the characteristic signs and symptoms of the disease
Interest over time
Google searches
Common signs & symptoms
Childhood onset
Cholangitis
Elevated C-reactive protein level
Epidermal acanthosis
Furrowed tongue
Geographic tongue
Current treatments
Generalized pustular psoriasis (GPP) is a severe form of psoriasis that typically requires aggressive treatment. Three current treatments for generalized pustular psoriasis include:
Systemic Retinoids
Oral retinoids such as acitretin are commonly used to treat GPP. Retinoids help to normalize skin cell growth and reduce inflammation. They are often effective in controlling pustular lesions and preventing flare-ups. However, retinoids can have significant side effects and require careful monitoring by a healthcare provider
Biologic Therapies
Biologic drugs, such as TNF-alpha inhibitors (e.g., adalimumab, infliximab) and IL-17 inhibitors (e.g., secukinumab, ixekizumab), have shown efficacy in treating GPP by targeting specific pathways involved in the inflammatory response. Biologics are typically reserved for cases of severe or refractory GPP that have not responded to other treatments
Cyclosporine
Cyclosporine is an immunosuppressive medication that can be used to rapidly control severe symptoms of GPP. It works by suppressing the immune system's activity, thereby reducing inflammation and preventing the formation of pustules. Cyclosporine is usually prescribed for short-term use due to the risk of adverse effects such as kidney damage and hypertension
Spesolimab (Spevigo)
The US Food and Drug Administration (FDA) has approved SPEVIGO® (spesolimab-sbzo) injection for the treatment of generalized pustular psoriasis (GPP) in adults and pediatric patients aged 12 and above weighing ≥40 kg. SPEVIGO® is a novel, humanized, selective antibody that specifically blocks the activation of the IL-36R, a signaling pathway within the immune system shown to be involved in the pathogenesis of several autoinflammatory diseases, including GPP