What are the treatment options for pustular psoriasis?

Treatment Options for Pustular Psoriasis

Generalized Pustular Psoriasis (GPP)

For generalized pustular psoriasis, first-line systemic therapies include acitretin, cyclosporine, methotrexate, and infliximab, with treatment selection based on disease severity and patient-specific contraindications. 1, 2

First-Line Systemic Therapies

• Acitretin is particularly effective for pustular psoriasis with response seen as early as 3 weeks at doses of 0.1-1 mg/kg/day 1, 2

  • Contraindicated in pregnancy or women planning pregnancy within 2 years of stopping treatment 1
  • Can be combined with narrowband UVB for synergistic effects 1

• Cyclosporine should be initiated at 2.5 mg/kg/day taken twice daily, with dose increases of 0.5 mg/kg/day every 2 weeks up to maximum 4 mg/kg/day if needed 1, 3

  • Contraindicated in abnormal renal function, uncontrolled hypertension, and previous/concomitant malignancy 1
  • Clinical improvement typically occurs within 2 weeks, with satisfactory control achieved in 12-16 weeks 3
  • Thirteen cases of transformation from chronic plaque psoriasis to pustular forms have been reported with cyclosporine 3

• Methotrexate is recommended for acute generalized pustular psoriasis, though specific dosing requires careful monitoring 1

  • Contraindicated in pregnancy, breastfeeding, significant hepatic damage, and blood disorders 1

• Infliximab has shown rapid efficacy in severe generalized pustular psoriasis with generally positive results including complete disease clearance 4, 1

  • Case reports demonstrate rapid response allowing withdrawal of conventional systemic treatments 4
  • Two of three patients in one follow-up study cleared completely with infliximab treatment 4

Second-Line Therapies

• Adalimumab and etanercept are considered second-line modalities for generalized pustular psoriasis 2

  • Etanercept at 50 mg biweekly (not 25 mg biweekly) showed clinical efficacy in a case series of 6 patients with maintenance of response for up to 48 weeks 4

• PUVA (psoralens plus ultraviolet A) is generally considered the systemic treatment of first choice with the least toxicity, achieving 45.7% efficacy in generalized pustular psoriasis 1

  • Contraindicated in pregnancy, significant cataracts, age <18, and previous cutaneous malignancy 1

Critical Management Considerations

• Avoid systemic corticosteroids due to risk of disease exacerbation upon discontinuation, though short-term therapy may be considered during severe systemic phases 1

  • Systemic corticosteroids can precipitate erythrodermic psoriasis, generalized pustular psoriasis, or very unstable psoriasis when discontinued 1
  • If a patient is experiencing a flare during steroid tapering, do not abruptly discontinue; instead initiate acitretin (0.1-1 mg/kg/day) while slowly tapering steroids 1

• Initial management usually requires hospital admission and systemic therapy for generalized pustular psoriasis 1

• Ultraviolet B radiation may be helpful but must be used with caution as it may exacerbate pustular psoriasis 1

Localized Pustular Psoriasis

Palmoplantar Pustulosis

• TNF antagonists should be avoided in chronic palmoplantar pustulosis 4

  • A pilot study found no benefit over placebo with etanercept 50 mg twice weekly for 12 weeks 4
  • Evidence suggests palmoplantar pustulosis is a distinct disease from psoriasis with different clinical and genetic profile 4
  • Increasing reports of new-onset palmoplantar pustulosis in patients treated with TNF antagonists for other conditions 4

• Moderately potent topical corticosteroids (grade III) are recommended for symptom relief in localized pustular psoriasis 1

  • Topical coal tar and dithranol provide some benefit 1

Acropustulosis (Acrodermatitis Continua of Hallopeau)

• TNF antagonists (etanercept, infliximab, adalimumab) are reasonable to recommend if acropustulosis has major impact on quality of life 4
  • At least 10 case reports show significant benefit from TNF antagonists for this rare but disabling condition 4
  • Only two reports of failure to respond, with one patient subsequently responding to a different TNF antagonist 4
  • Frequently unresponsive to conventional systemic antipsoriatic agents 4

Emerging Therapies

• Spesolimab (IL-36 receptor inhibitor) has shown promising results in severe GPP flares resistant to conventional treatments 5, 6
  • Administered as 900 mg infusions, can lead to swift improvement with resolution of pustules and skin inflammation 6
  • Represents targeted therapy for IL-36-mediated disease pathophysiology 5, 6

Monitoring Requirements

• All systemic treatments require appropriate pretreatment assessment and ongoing monitoring with regular blood tests, liver function tests, and serum creatinine monitoring depending on the agent used 1

• Combination therapy should be approached with extreme caution as toxicity is at least additive 1

• Long-term continuous treatment for extended periods greater than one year is not recommended for cyclosporine in psoriasis patients; alternation with other forms of treatment should be considered 3