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Generalized Pustular Psoriasis (GPP)

Severe psoriasis subtype with sterile pustules: emergency biologic treatment

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dermatoloji department. Book Appointment →

What is Generalized Pustular Psoriasis (GPP)?

GPP is a rare condition (incidence 1-2/million) classified by ERASPEN criteria; the von Zumbusch type is the acute, severe form. Fever (>38°C), erythroderma, sterile pustules, leukocytosis, hypoalbuminemia, and hypocalcemia are the dominant findings. Mortality is 2-30% (cardiac, pulmonary, septic complications).

Pathogenesis: IL-36 cytokine pathway plays a central role; loss-of-function mutation in IL36RN gene (DITRA syndrome - 25%), CARD14, AP1S3, and MPO mutations have been identified. Th17 axis (IL-17, IL-23) is also activated. Triggering factors: infection, pregnancy, hypocalcemia, oral steroid withdrawal, drugs.

Treatment has historically used systemic retinoids, MTX, cyclosporine, biologics (IL-17, IL-23). The IL-36 receptor antagonist spesolimab (FDA approval 2022) provides 90% reduction in pustules at 1 week and continued improvement at 12 weeks; imsidolimab and other IL-36 inhibitors are in development.

Symptoms

Acute high fever (>38.5°C)
Diffuse erythema and sterile pustules (1-2 mm)
Pustule lakes (fusing together)
Burning, pain, severe itching on skin
Generalized weakness, malaise
Hypocalcemia and hypoalbuminemia (severe inflammation)

Risk Factors

Genetic mutation (IL36RN/DITRA, CARD14)
Pregnancy (impetigo herpetiformis)
Sudden withdrawal of oral steroids
Infection (especially streptococcus)
Plaque psoriasis history
Hypocalcemia and hypoparathyroidism

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Sudden onset diffuse erythema and pustules
  • Fever and systemic findings (sepsis suspicion)
  • Pustular eruption developing during pregnancy
  • Pustular eruption after steroid withdrawal
  • Pustule appearance on plaque psoriasis
  • Erythroderma + cardiac/pulmonary symptoms

Treatment Methods

01
Spesolimab (IL-36R antagonist) - flare treatment, 1 week response
02
Cyclosporine (3-5 mg/kg/day) - rapid response, 1st line option
03
Acitretin (0.5-1 mg/kg/day) - chronic management
04
IL-17 inhibitor (secukinumab, brodalumab)
05
IL-23 inhibitor (guselkumab, risankizumab)
06
Supportive: fluid, electrolyte, calcium, infection control

Which Department to Visit?

You can visit our Dermatoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.