pustular psoriasis; impetigo herpetiformis, von Zumbusch's disease; acrodermatitis continua of Hallopeau

Etiology

• generalized exacerbation of psoriasis due to glucocorticoid withdrawal
• impetigo herpetiformis is a form of severe pustular psoriasis that occurs in pregnancy
• infection
• chemical exposure, medications

Epidemiology

• generalized form seen in adults

Pathology

• generalized or localized pustular psoriasis, active stage
• acrodermatitis continua of Hallopeau affects hands & feet
• spongiform pustules of Kogoj (collections of neutrophils in spiny & granular layers

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Clinical manifestations

• repeated flares of sudden onset consisting of diffuse erythematous skin eruption characterized by rapid coveragewith pustules, high-grade fever, asthenia
• scaling, sheets of superficial pustules, erosions
• lakes of sterile pus & reddened skin that often itch & burn
• may present alone, or may present with other sites with plaque psoriasis

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Laboratory

• complete blood count
  • leukocytosis

Complications

• potentially life-threatening disease

Differential diagnosis

• skin infection

Management

• generalized pustular psoriasis usually requires hospitalization for supportive care & systemic therapy
  • affected sites are usually too large to manage with topical medications alone
• refer to dermatologist
• impetigo herpetiformis is the only form of pustular psoriasis treated with glucocorticoids
• IL-36 inhibitor spesolimab (investigational)
  • reduces flaes of pustular psoriasis

More general terms

• psoriasis
• dermatoses of pregnancy