(von Zumbusch) acute generalized pustular psoriasis

Acute generalized pustular psoriasis (von Zumbusch)
Classification and external resources
ICD-10 L40.1

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]

Characteristics

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. ISSN 0305-4179. doi:10.1016/j.burns.2014.01.003.
  2. 1 2 3 4 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. ISSN 1879-1409. PMID 24491419. doi:10.1016/j.burns.2014.01.003.
  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. ISSN 1365-4632. PMID 23967807. doi:10.1111/ijd.12070.
  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. ISSN 1097-6787. PMID 22609220. doi:10.1016/j.jaad.2011.01.032.
  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. ISSN 1538-3652. PMID 23247492. doi:10.1001/2013.jamadermatol.80.
  7. ↑ Zumbusch, Leo Ritter von (1909-02-01). "Psoriasis und pustulöses Exanthem". Archiv für Dermatologie und Syphilis (in German). 99 (1-2): 335–346. ISSN 0365-6020. doi:10.1007/BF01910970.
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