Generalized pustular psoriasis - a model disease for specific targeted immunotherapy, systematic review

Area of Science:

• Dermatology
• Immunology
• Precision Medicine

Background:

• Generalized pustular psoriasis (GPP) is a rare, severe, and heterogeneous multisystemic skin disease.
• GPP presents significant challenges in diagnosis and treatment, necessitating better disease stratification.
• Understanding GPP pathogenesis is crucial for developing effective precision medicine approaches.

Purpose of the Study:

• To systematically review literature on the efficacy of targeted immunotherapies for adult GPP.
• To analyze the mode of action of these therapies in relation to GPP phenotype, classification, and pathogenesis.
• To evaluate treatment response rates based on defined diagnostic criteria.

Main Methods:

• Systematic literature review of 101 published adult GPP cases.
• Inclusion criteria: compatible clinical history, dermatological features/histopathology, consistent clinical picture, and DITRA status.
• Identification of targeted immunotherapies (TNF-α, IL-12/23, IL-17, IL-1β) and assessment of treatment outcomes.

Main Results:

• Targeted immunotherapies demonstrated significant efficacy in treating GPP.
• 61% of patients achieved complete response, and 27% achieved partial response.
• Anti-interleukin-17 (anti-IL-17) agents showed the most substantial evidence of effectiveness.

Conclusions:

• Targeted immunotherapy is an effective treatment strategy for generalized pustular psoriasis.
• The study highlights the critical role of IL-17, IL-1, and IL-36 pathways in GPP pathogenesis.
• Further research and standardized classification are needed to optimize GPP management.