Просмотр кода страницы Английская Википедия:(von Zumbusch) acute generalized pustular psoriasis

{{Английская Википедия/Панель перехода}}
'''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.<ref>{{Cite journal|last1=Varman|first1=Katherine M.|last2=Namias|first2=Nicholas|last3=Schulman|first3=Carl I.|last4=Pizano|first4=Louis R.|date=2014-06-01|title=Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics|url=http://www.burnsjournal.com/article/S0305-4179(14)00016-3/abstract|journal=Burns|language=English|volume=40|issue=4|pages=e35–e39|doi=10.1016/j.burns.2014.01.003|pmid=24491419|issn=0305-4179}}</ref>

== 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 [[Erythrocyte sedimentation rate|ESR]], elevated [[C-reactive protein|CRP]], combined with leukocytosis).<ref name=":0">{{Cite book|title=Rook's textbook of dermatology|last1=Griffiths|first1=C|last2=Barker|first2=Jonathan|last3=Bleiker|first3=Tanya|last4=Chalmers|first4=Robert|last5=Creamer|first5=Daniel|year=2016|publisher=John Wiley & Sons Inc|isbn=9781118441190|oclc=949329582}}</ref>

== Diagnosis ==
Kogoj's spongiform pustules can be observed via histopathology to confirm acute GPP.<ref name=":0" />

== Treatment ==
Acute GPP typically requires inpatient management including both topical and systemic therapy, and supportive measures.<ref>{{Cite journal|last1=Varman|first1=Katherine M.|last2=Namias|first2=Nicholas|last3=Schulman|first3=Carl I.|last4=Pizano|first4=Louis R.|date=2014-06-01|title=Acute generalized pustular psoriasis, von Zumbusch type, treated in the burn unit. A review of clinical features and new therapeutics|journal=Burns: Journal of the International Society for Burn Injuries|volume=40|issue=4|pages=e35–39|doi=10.1016/j.burns.2014.01.003|issn=1879-1409|pmid=24491419}}</ref> Systemic glucocorticoid withdrawal is a common causative agent.<ref>{{Cite journal|last1=Choon|first1=Siew Eng|last2=Lai|first2=Nai Ming|last3=Mohammad|first3=Norshaleyna A.|last4=Nanu|first4=Nalini M.|last5=Tey|first5=Kwee Eng|last6=Chew|first6=Shang Fern|date=2014-06-01|title=Clinical profile, morbidity, and outcome of adult-onset generalized pustular psoriasis: analysis of 102 cases seen in a tertiary hospital in Johor, Malaysia|journal=International Journal of Dermatology|volume=53|issue=6|pages=676–684|doi=10.1111/ijd.12070|issn=1365-4632|pmid=23967807|s2cid=44470715}}</ref> 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.<ref name=":0" /> Cyclosporine or infliximab may be required for particularly acute cases.<ref>{{Cite journal|last1=Robinson|first1=Amanda|last2=Van Voorhees|first2=Abby S.|last3=Hsu|first3=Sylvia|last4=Korman|first4=Neil J.|last5=Lebwohl|first5=Mark G.|last6=Bebo|first6=Bruce F.|last7=Kalb|first7=Robert E.|date=2012-08-01|title=Treatment of pustular psoriasis: from the Medical Board of the National Psoriasis Foundation|journal=Journal of the American Academy of Dermatology|volume=67|issue=2|pages=279–288|doi=10.1016/j.jaad.2011.01.032|issn=1097-6787|pmid=22609220}}</ref><ref>{{Cite journal|last1=Viguier|first1=Manuelle|last2=Aubin|first2=François|last3=Delaporte|first3=Emmanuel|last4=Pagès|first4=Cécile|last5=Paul|first5=Carle|last6=Beylot-Barry|first6=Marie|last7=Goujon|first7=Catherine|last8=Rybojad|first8=Michel|last9=Bachelez|first9=Hervé|date=2012-12-01|title=Efficacy and safety of tumor necrosis factor inhibitors in acute generalized pustular psoriasis|journal=Archives of Dermatology|volume=148|issue=12|pages=1423–1425|doi=10.1001/2013.jamadermatol.80|issn=1538-3652|pmid=23247492|doi-access=free}}</ref>

== History ==
The disorder has been named after Leo Ritter von Zombusch, who first described two cases of a brother and a sister in 1910.<ref>{{Cite journal|last=Zumbusch|first=Leo Ritter von|date=1909-02-01|title=Psoriasis und pustulöses Exanthem|journal=Archiv für Dermatologie und Syphilis|language=de|volume=99|issue=1–2|pages=335–346|doi=10.1007/BF01910970|s2cid=11086906|issn=0365-6020|url=https://zenodo.org/record/2155152}}</ref> The patients experienced patterns of redness and pustule formation over several years, often associated with use of topical medications.<ref name=":0" /> Unfortunately one of the two siblings died from complications of the disease.

== References ==
{{Reflist}}
== External links ==
{{Medical resources
|  ICD10 = L40.1
}}
[[Category:Psoriasis]]
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