(von Zumbusch) acute generalized pustular psoriasis

von Zumbusch (acute) generalized pustular psoriasis (acute GPP) is the most severe form of generalized pustular psoriasis, and can be associated with life-threatening complications.[1]

Signs and symptoms

Patients with acute GPP experience the eruption of multiple isolated sterile pustules generalized over the body, recurrent fevers, fatigue, and laboratory abnormalities (elevated ESR, elevated CRP, combined with leukocytosis).[2]

Diagnosis

Kogoj's spongiform pustules can be observed via histopathology to confirm acute GPP.[2]

Treatment

Acute GPP typically requires inpatient management including both topical and systemic therapy, and supportive measures.[3] Systemic glucocorticoid withdrawal is a common causative agent.[4] Withdrawal or administration of certain drugs in the patient's previous medication regimen may be required. Oral retinoids are the most effective treatment, and are considered first line.[2] Cyclosporine or infliximab may be required for particularly acute cases.[5][6]

History

The disorder has been named after Leo Ritter von Zombusch, who first described two cases of a brother and a sister in 1910.[7] The patients experienced patterns of redness and pustule formation over several years, often associated with use of topical medications.[2] Unfortunately one of the two siblings died from complications of the disease.

References
  1. Varman, Katherine M.; Namias, Nicholas; Schulman, Carl I.; Pizano, Louis R. (2014-06-01). "Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics". Burns. 40 (4): e35–e39. doi:10.1016/j.burns.2014.01.003. ISSN 0305-4179. PMID 24491419.
  2. Griffiths, C; Barker, Jonathan; Bleiker, Tanya; Chalmers, Robert; Creamer, Daniel (2016). Rook's textbook of dermatology. John Wiley & Sons Inc. ISBN 9781118441190. OCLC 949329582.
  3. Varman, Katherine M.; Namias, Nicholas; Schulman, Carl I.; Pizano, Louis R. (2014-06-01). "Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics". Burns: Journal of the International Society for Burn Injuries. 40 (4): e35–39. doi:10.1016/j.burns.2014.01.003. ISSN 1879-1409. PMID 24491419.
  4. Choon, Siew Eng; Lai, Nai Ming; Mohammad, Norshaleyna A.; Nanu, Nalini M.; Tey, Kwee Eng; Chew, Shang Fern (2014-06-01). "Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia". International Journal of Dermatology. 53 (6): 676–684. doi:10.1111/ijd.12070. ISSN 1365-4632. PMID 23967807. S2CID 44470715.
  5. Robinson, Amanda; Van Voorhees, Abby S.; Hsu, Sylvia; Korman, Neil J.; Lebwohl, Mark G.; Bebo, Bruce F.; Kalb, Robert E. (2012-08-01). "Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation". Journal of the American Academy of Dermatology. 67 (2): 279–288. doi:10.1016/j.jaad.2011.01.032. ISSN 1097-6787. PMID 22609220.
  6. Viguier, Manuelle; Aubin, François; Delaporte, Emmanuel; Pagès, Cécile; Paul, Carle; Beylot-Barry, Marie; Goujon, Catherine; Rybojad, Michel; Bachelez, Hervé (2012-12-01). "Efficacy and safety of tumor necrosis factor inhibitors in acute generalized pustular psoriasis". Archives of Dermatology. 148 (12): 1423–1425. doi:10.1001/2013.jamadermatol.80. ISSN 1538-3652. PMID 23247492.
  7. Zumbusch, Leo Ritter von (1909-02-01). "Psoriasis und pustulöses Exanthem". Archiv für Dermatologie und Syphilis (in German). 99 (1–2): 335–346. doi:10.1007/BF01910970. ISSN 0365-6020. S2CID 11086906.
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