From the Guidelines
Pustular psoriasis should be treated with infliximab for rapid control, as it has shown generally positive results in severe cases, with some patients achieving complete disease clearance 1.
Treatment Overview
Pustular psoriasis is a rare and severe form of psoriasis characterized by the development of sterile pustules on red, inflamed skin. The treatment approach typically involves a combination of topical and systemic therapies. For mild cases, treatment may begin with topical corticosteroids like clobetasol 0.05% applied twice daily, combined with calcipotriene (a vitamin D analog) for enhanced efficacy.
Systemic Treatments
For more extensive disease, systemic treatments are necessary. Acitretin, starting at 25-50mg daily, is often used as first-line therapy. In severe or rapidly progressing cases, cyclosporine (2.5-5mg/kg/day) provides rapid control, though its use is limited to short-term due to side effects. Biologics like IL-17 inhibitors (secukinumab, ixekizumab) or IL-23 inhibitors (guselkumab, risankizumab) are highly effective for resistant cases.
Generalized Pustular Psoriasis
Generalized pustular psoriasis may require hospitalization for IV fluids, infection monitoring, and possibly methotrexate (10-25mg weekly) or infliximab for rapid control, as supported by guidelines and case reports 1. Infliximab has been particularly noted for its efficacy in this context, with reports of complete disease clearance in some patients.
Monitoring and Precautions
The condition results from dysregulated inflammatory pathways, particularly involving IL-1 and IL-36, leading to neutrophil recruitment and pustule formation. Regular monitoring of liver function, blood counts, and kidney function is essential during systemic therapy. Patients should avoid triggers like certain medications, infections, and sudden steroid withdrawal to prevent exacerbations.
Key Considerations
- Infliximab is recommended for severe cases of generalized pustular psoriasis due to its rapid efficacy and potential for complete disease clearance 1.
- Methotrexate can be used for acute generalized pustular psoriasis and as a long-term maintenance treatment, with careful monitoring for side effects like marrow suppression and hepatic fibrosis 1.
- Biologics offer effective treatment options for resistant cases, targeting specific inflammatory pathways involved in psoriasis.
From the FDA Drug Label
There were 9 cases of pustular and 4 cases of erythrodermic psoriasis. Thirteen cases of transformation of chronic plaque psoriasis to more severe forms of psoriasis have been reported In clinical trials, cyclosporine doses at the lower end of the recommended dosage range were effective in maintaining a satisfactory response in 60% of the patients. Results of a dose-titration clinical trial with cyclosporine [MODIFIED] indicate that an improvement of psoriasis by 75% or more (based on PASI) was achieved in 51% of the patients after 8 weeks and in 79% of the patients after 16 weeks.
Pustular Psoriasis Treatment with Cyclosporine:
- The drug label reports 9 cases of pustular psoriasis in patients treated with cyclosporine.
- Cyclosporine can be effective in treating psoriasis, with 51% of patients achieving a 75% or more improvement after 8 weeks, and 79% after 16 weeks.
- However, the label also notes that long-term experience with cyclosporine in psoriasis patients is limited, and continuous treatment for extended periods greater than one year is not recommended 2.
From the Research
Definition and Classification of Pustular Psoriasis
- Pustular psoriasis is a group of skin diseases characterized by neutrophil infiltration in the epidermis and formation of sterile pustules 3
- It has been classified into localized and generalized forms, with generalized pustular psoriasis (GPP) being a rare, severe neutrophilic skin disorder 4
- Localized forms include palmoplantar pustulosis (PPP) and acrodermatitis continua of Hallopeau (ACH) 5
Treatment Options for Pustular Psoriasis
- Treatment should be governed by the extent of involvement and severity of disease, with acitretin, cyclosporine, methotrexate, and infliximab considered first-line therapies for generalized pustular psoriasis 6
- Adalimumab, etanercept, and psoralen plus ultraviolet A are considered second-line modalities for generalized pustular psoriasis 6
- Biologics, including anti-TNF-α, anti-IL-12/23, and anti-IL-17A, have emerged as potential treatments for pustular psoriasis, with several being approved in Japan 3, 4
- Infliximab has been shown to be efficacious for the long-term management of GPP, particularly when used in combination with methotrexate 7
Challenges and Considerations in Treating Pustular Psoriasis
- The quality of the literature about the treatment of pustular psoriasis is weak, with few high-quality studies examining treatment options 6
- Treatment of patients with pustular psoriasis depends on the severity of presentation and patient's underlying risk factors, with concerns such as teratogenicity needing to be factored into decision-making 6
- Corticosteroids should be avoided in the management of any form of psoriasis, as they can trigger and lead to flares of GPP 5