International Psoriasis Council

Advancing Knowledge. Improving Care.

Advancing Knowledge. Improving Care.

New IPC Guidelines on Generalized Pustular Psoriasis

Generalized Pustular Psoriasis (GPP) is a rare and potentially life-threatening form of psoriasis that can present at any age, but the median age is around 50. GPP is characterized by widespread areas of inflamed skin with pustules, and its severity can fluctuate, with periods of flare-ups followed by remission. Flares may be triggered by multiple factors, such as rapid withdrawal of systemic corticosteroids, infections, pregnancy, and even stress. The etiology of GPP is not fully understood, but emerging evidence implicates a multifactorial interplay of genetic, environmental, and immune dysregulation factors. Genetic predisposition, particularly mutations in genes such as IL36RN, highlights the crucial role of the IL-36 pathway in the pathogenesis of GPP.

 Currently, GPP is treated with non-biologics and biologics approved for treating psoriasis. The only approved treatment option for GPP is spesolimab, an IL-36 receptor antibody. Intravenous spesolimab is approved for flares in some countries (e.g., the United States, Japan, China, Taiwan, and Canada). Meanwhile, subcutaneous spesolimab has been approved for flare prevention in the United States and China. Other treatment options include non-biologic system therapies. Finally, the role of the IL-36 pathway as a key inflammatory factor in GPP is driving the development of new targeted treatments.

Recently, the International Psoriasis Council (IPC) made significant strides by publishing a consensus statement that aims to standardize the diagnosis of GPP globally. 

Key Takeaways from the IPC's New GPP Diagnostic Criteria:

  1. Acral Distinction: Unlike other consensuses, the IPC recognizes that pustular lesions on the palms and soles can occur during GPP flares but should not rule out a GPP diagnosis alone. This acknowledgment is crucial as it addresses the coexistence of GPP with other localized forms of pustular psoriasis, ensuring appropriate and urgent treatment.
  2. Incorporation of Erythema: The new criteria identify erythema as an essential feature of GPP. This addition is vital as erythema indicates the inflammatory nature of the disease. Nearly 80% of the reviewed GPP cases involved individuals with darker skin tones, where erythema might be less discernible yet still significant.
  3. Implementation of Criteria: For dermatologists suspecting GPP, thorough patient history and physical examination, guided by the new criteria, is paramount. The criteria also recommend specific steps like a biopsy to confirm the diagnosis, particularly when distinguishing GPP from other dermatoses like AGEP and SPD.
  4. Role of Biopsy and Direct Immunofluorescence (DIF): While biopsy/histopathology remains highly recommended for confirming GPP, the role of DIF in diagnosis appears limited, with only a minor consensus supporting its utility in ruling out other skin conditions.
  5. Utility Across Medical Disciplines: These criteria are designed to be used by dermatologists and general practitioners. This broader applicability ensures timely referrals and appropriate care, potentially improving patient outcomes.

Impact on Global Health Practices

The broad representation in the study, with 78% of cases being individuals with darker skin tones, ensures that the criteria are inclusive and applicable globally. This diversity addresses previous biases toward lighter skin presentations, enhancing diagnostic accuracy and treatment across various ethnicities and geographic areas.

Conclusion

The IPC’s consensus on GPP is a landmark development in dermatology, promising to standardize the approach to diagnosing and treating this challenging condition worldwide. As these criteria are adopted and implemented, we anticipate significant improvements in patient care and outcomes, particularly for those in underrepresented populations or with severe disease manifestations. This initiative enhances our understanding of GPP and exemplifies the power of global collaboration in health care.

We look forward to seeing the positive changes this new consensus will bring to the lives of patients with generalized pustular psoriasis worldwide. Read the complete consensus statement here.

References

International Consensus Definition and Diagnostic Criteria for Generalized Pustular Psoriasis from the International Psoriasis Council. Choon SE, van de Kerkhof P, Gudjonsson JE, et al. JAMA Dermatol. Published online May 01, 2024. doi:10.1001/jamadermatol.2024.0915

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