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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 * image [7] 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 * images [4,5,7] 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 [6] - 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 [8]

General

psoriasis dermatoses of pregnancy

References

  1. Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
  2. Wikipedia: Impetigo herpetiformis http://en.wikipedia.org/wiki/Impetigo_herpetiformis
  3. Oumeish OY, Parish JL. Impetigo herpetiformis. Clin Dermatol. 2006 Mar-Apr;24(2):101-4. PMID: 16487882
  4. DermNet NZ. Generalised pustular psoriasis (images) http://www.dermnetnz.org/scaly/pustular-psoriasis.html
  5. Morrison AO, Elston DM (images) Medscape: Pustular Psoriasis Overview of Pustular Psoriasis http://emedicine.medscape.com/article/1108220-overview
  6. Meffert J (image) Psoriasis: Manifestations, Management Options, and Mimics. Medscape. Dec 17,, 2015 http://reference.medscape.com/features/slideshow/psoriasis-subtypes
  7. Camilleri MJ, Link JL Pustular Psoriasis Mayo Clinic Proceedings. 2022. 97(12):2385-86 https://www.mayoclinicproceedings.org/article/S0025-6196(22)00509-2/fulltext
  8. Morita A, Strober B, Burden AD, et al. Efficacy and safety of subcutaneous spesolimab for the prevention of generalised pustular psoriasis flares (Effisayil 2): an international, multicentre, randomised, placebo-controlled trial. Lancet. 2023 Sep 19:S0140-6736(23)01378-8 PMID: 37738999 https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(23)01378-8/fulltext