GENERALIZED PUSTULAR PSORIASIS

Generalized Pustular Psoriasis (GPP) is a severe, potentially life-threatening inflammatory dermatosis characterized by diffuse erythema and pustules.

• GPP pathogenesis primarily involves abnormal activation of the interleukin (IL)-36 pathway.
• Fever, generalized erythema, and pustules develop acutely. Adults are typical, but infants and children may be affected.
• Tar may precipitate or exacerbate GPP and should be avoided.
  
• A geographic or fissured tongue may occur.
• Blood cultures may be indicated to exclude infection.
  

Although the majority of patients do not have genetic mutations, mutations in the following genes have been found; interleukin-36 receptor antagonist, caspase recruitment domain family member 14, adapter related protein complex 1 subunit sigma, serpin family A member 3, and myeloperoxidase.

THe patient presents acutely wiht a diffuse eruption of erythematous plaques with pustules at the periphery. It is typical to have fever (e.g to 104 F), chills, tachycardia and leukocytosis.

Triggers

Steroid administration and classically steroid withdrawal can precipitate GPP. A strep throat infection may trigger the disease or precipitate a flare. Pregnancy as well as various drugs have been reported to precipitate GPP including calcipotriene, tar, beta-blockers, TNF-alpha inhibitors, NSAIDs, and lithium. Generalized pustular psoriasis has developed in patients following withdrawal of cyclosporin for treatment of palmar plantar pustulosis.

RegionalDerm

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