257:. They describe the pattern as having "waves of widespread or universally fiery redness". The affected areas are "painful and tender". Small sub-corneal pustules form, with sizes originally between 1 and 10 mm in diameter. These pustules may merge to form "yellow-green lakes of pus". The pustules dry out, and "Waves of scarlatiniform peeling follow, removing the desiccating pustules". In regards to the onset, the von Zumbusch form may "supervene on any previous pattern of psoriasis". It also may or may not recur periodically.
50:
250:(1874–1940), son of Kaspar von Zumbusch, who described the first documented case of generalized pustular psoriasis in the early 1900s. See Case Report #1. Sometimes all or any of GPP is referred to as von Zumbusch psoriasis, but in the literature it is often distinguished as one specific form of GPP.
798:
Mahil, SK; Twelves, S; Farkas, K; Setta-Kaffetzi, N; Burden, AD; Gach, JE; Irvine, AD; Képíró, L; Mockenhaupt, M; Oon, HH; Pinner, J; Ranki, A; Seyger, MM; Soler-Palacin, P; Storan, ER; Tan, ES; Valeyrie-Allanore, L; Young, HS; Trembath, RC; Choon, SE; Szell, M; Bata-Csorgo, Z; Smith, CH; Di Meglio,
315:
According to the article by the
University of São Paulo, mentioned above, "The onset of childhood GPP is generally abrupt and accompanied by toxic features." The original acute episode usually lasts a few days, but "repeated waves of inflammation and pustulation may follow." It is important that the
109:
The
Department of Dermatology of the University of São Paulo proposed a classification for these two conditions. Pso+ represents patients with a personal history of psoriasis and pso- represents patients with no history of psoriasis. They also identified a common factor among patients in each group:
70:
that can present in a variety of forms. Unlike the most general and common forms of psoriasis, GPP usually covers the entire body and with pus-filled blisters rather than plaques. GPP can present at any age, but is rarer in young children. It can appear with or without previous psoriasis conditions
433:
Hazarika gave a report of a 29-year-old woman with no family history of psoriasis, having had a normal first pregnancy, who presented with GPP in the twenty-eighth week of her second pregnancy. Steroid therapy caused a worsening of the symptoms. With cyclosporine the lesions cleared in 10–14 days,
307:
by the
Department of Dermatology, University of São Paulo. The report acknowledged that psoriasis is a relatively common skin condition in children, but "the pustular variant is rare." Out of 1,262 cases of psoriasis in children, a "0.6% rate of pustular variants" was found. When GPP does occur in
410:
said, "The GPP pattern is as an acute, episodic, and potentially life-threatening form of psoriasis." There is no cure-all treatment for GPP, and as such, the mortality rate is high. Ryan and Baker study from 1971 observed 155 patients with GPP, 106 of which were followed up with. 26 of those 106
714:
Marrakchi S, Guigue P, Renshaw BR, Puel A, Pei XY, Fraitag S, Zribi J, Bal E, Cluzeau C, Chrabieh M, Towne JE, Douangpanya J, Pons C, Mansour S, Serre V, Makni H, Mahfoudh N, Fakhfakh F, Bodemer C, Feingold J, Hadj-Rabia S, Favre M, Genin E, Sahbatou M, Munnich A, Casanova JL, Sims JE, Turki H,
462:
A case report published in the
Journal of Dermatological Treatment documents the successful use of adalimumab to control symptoms and induce relapse for 72 weeks. "Adalimumab is ... approved for the treatment of moderate to severe rheumatoid arthritis ... and more recently for the treatment of
113:
In a large portion of cases, the disease is brought on by some triggering factor. Through research and observation, many of these factors have been identified. The following table, from an article in Cutis, lists a few factors that have been observed as influential in the onset of GPP.
424:
Von
Zumbusch observed a male patient, who had had classic psoriasis for several years, and who then went through recurrent episodes of bright and , which became studded with multiple pustules. Von Zumbusch observed this patient through nine hospital admissions over 10 years.
275:
In 2009, Dr. Debeeka
Hazarika, president of the North East States branch of the Indian Association of Dermatologists, Venereologists and Leprologists (IADVL), published an article titled "Generalized pustular psoriasis of pregnancy successfully treated with cyclosporine" in
316:
disease is managed immediately in order to prevent life-threatening complications, such as infection or . Other complications include "metabolical, hemo-dynamic, and thermoregulatory disturbances" which occur as a result of "alterations of the epidermal barrier."
327:
This type of psoriasis appears as round lesions. It begins as discrete areas that become raised and swollen. Pustules appear at the edges of the round lesions, creating rings. The pustules then dry out and leave a trail of scale as the lesion grows.
756:
Berki, DM; Liu, L; Choon, SE; David Burden, A; Griffiths, CEM; Navarini, AA; Tan, ES; Irvine, AD; Ranki, A; Ogo, T; Petrof, G; Mahil, SK; Duckworth, M; Allen, MH; Vito, P; Trembath, RC; McGrath, J; Smith, CH; Capon, F; Barker, JN (December 2015).
1159:
339:
Treatments vary widely, and many different drugs have been documented as being successful. Some medications are successful in some patients, while unsuccessful in others. Below is a list of some medications used to treat GPP:
311:
Khan et al. reported that in GPP patients ten or younger, less than 12% of cases are preceded by ordinary psoriasis. This differs greatly from GPP cases in adults, where 85% of GPP is preceded by typical psoriatic lesions.
98:, is "sheeted, pinhead-sized, sterile, sub-corneal pustules". The IPC roundtable adds that these pustules often occur either at the edges "of expanding, intensely inflammatory plaques" or "within erythrodermic skin".
271:
This form of GPP tends to have symmetrical and grouped features. It usually onsets early in the third trimester of pregnancy, and generally persists until the child is born, but occasionally long after.
434:
but new lesions appeared. The patient gave birth to a healthy baby in the thirty-eighth week of pregnancy. A month and a half after delivery, the woman presented with a psoriatic plaque on her leg.
106:
Most cases of generalized pustular psoriasis present in patients with existing or prior psoriasis conditions. However, there are many cases of GPP that arise without a history of psoriasis.
454:. After receiving a partial thickness burn from overexposure, he presented with annular pustular psoriasis, which cleared after 21 days, only to reoccur every 3 to 6 weeks for a year.
442:
An eleven-year-old boy had an eight-year history of recurrent GPP. He suffered from "fever, malaise and pain". He was treated with acitretin, and improvement was seen in five weeks.
593:, Christophers E, Barker JN, Chalmers RJ, Chimenti S, Krueger GG, Leonardi C, Menter A, Ortonne JP, Fry L (2007). "A classification of psoriasis vulgaris according to phenotype".
232:
It is important to note that while there are different forms of GPP, they are not exclusive of each other. One can morph into another, or multiple forms can occur simultaneously.
241:
406:
GPP is a rare and severe type of psoriasis. It in rare cases it is said to be fatal and in some cases has driven patients to intensive burn units. An article published in
110:
In the pso+ group, the most common precipitating factor is corticosteroid withdrawal. In the pso- group, the most common precipitating factor is infection.
283:
In 1979, Frank R. Murphy, MD and Lewis P. Stolman, MD reported on the case of a woman who developed generalized pustular psoriasis in response to doses of
1027:
Pomahac B, Lim J, Liu A (2008). "A case report of generalized pustulosis with systemic manifestations requiring burn intensive care unit admission".
759:"Activating CARD14 Mutations Are Associated with Generalized Pustular Psoriasis but Rarely Account for Familial Recurrence in Psoriasis Vulgaris"
280:. As reported by Hazarika, there have been up to nine instances where the disease was recurrent in subsequent pregnancies. See Case Report 2
550:
Khan SA, Peterkin GA, Mitchell PC (1972). "Juvenile generalized pustular psoriasis. A report of five cases and a review of the literature".
208:
938:
Zangrilli A, Papoutsaki M, Talamonti M, Chimenti S (2008). "Long-term efficacy of adalimumab in generalized pustular psoriasis".
590:
643:
de
Oliveira ST, Maragno L, Arnone M, Fonseca Takahashi MD, Romiti R (2010). "Generalized pustular psoriasis in childhood".
253:
Eugene M. Farber, MD and colleagues provide a description of von
Zumbusch psoriasis in "Pustular Psoriasis", published in
472:
207:
Although there are likely to be multiple genetic factors and environmental triggers, mutations causing defects in the
801:"AP1S3 Mutations Cause Skin Autoinflammation by Disrupting Keratinocyte Autophagy and Up-Regulating IL-36 Production"
87:
over a wide area of the body. It differs from the localized form of pustular psoriasis in that patients are often
266:
900:
Zelickson BD, Muller SA (1991). "Generalized pustular psoriasis in childhood. Report of thirteen cases".
854:
291:
and GPP. Most cases of GPP in pregnancy occur late in the third trimester, generally when production of
450:
In 1991, a case was reported of a man having plaque psoriasis and treating it with UV radiation at a
303:
GPP is a rare disease in general, but even more so in children. In 2010, an article was published in
126:
84:
49:
17:
1191:
1087:
963:
618:
162:
1122:
1105:
Rosen RM (1991). "Annular pustular psoriasis induced by UV radiation from tanning salon use".
1079:
1044:
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879:
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610:
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38:
1114:
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1036:
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947:
909:
869:
820:
812:
770:
728:
652:
602:
559:
517:
191:
411:
died as a result of the psoriasis or the treatment. Their data gives a 25% mortality rate.
216:
365:
563:
521:
1075:
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800:
150:
142:
1118:
1185:
913:
656:
606:
370:
1091:
967:
855:"Generalized pustular psoriasis of pregnancy successfully treated with cyclosporine"
622:
385:
292:
146:
138:
717:"Interleukin-36-receptor antagonist deficiency and generalized pustular psoriasis"
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505:
451:
354:
288:
43:
951:
816:
380:
375:
344:
158:
134:
1062:
Ryan TJ, Baker H (1971). "The prognosis of generalized pustular psoriasis".
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67:
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834:
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529:
170:
80:
1151:
395:
359:
130:
88:
1163:
212:
261:
Generalized pustular psoriasis of pregnancy (Impetigo herpetiformis)
1168:
349:
246:
Von
Zumbusch psoriasis is named after the German dermatologist
199:
Source: "Table II", "Pustular
Psoriasis" Farber and Nall, 1993
308:
children, it usually appears during the first year of life.
94:
However, the most prominent symptom, as described in the
862:
Indian Journal of Dermatology, Venereology and Leprology
1141:
1145:
242:(von Zumbusch) acute generalized pustular psoriasis
120:Provocative Factors Influencing Pustular Psoriasis
37:
32:
71:or history, and can reoccur in periodic episodes.
236:von Zumbusch acute generalized pustular psoriasis
687:Farber EM, Nall L (1993). "Pustular psoriasis".
1107:Journal of the American Academy of Dermatology
902:Journal of the American Academy of Dermatology
8:
1142:
48:
29:
1003:
988:"Juvenile generalized pustular psoriasis"
873:
824:
799:P; Barker, JN; Capon, F (November 2016).
774:
732:
805:The Journal of Investigative Dermatology
763:The Journal of Investigative Dermatology
116:
489:
181:Infections: dental, upper respiratory
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564:10.1001/archderm.1972.01620040039007
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522:10.1001/archderm.1979.04010100035015
499:
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495:
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219:genes have been shown to cause GPP.
940:Journal of Dermatological Treatment
1076:10.1111/j.1365-2133.1971.tb14044.x
25:
18:Pustular psoriasis of von Zumbusch
278:Indian J Dermatol Venereol Leprol
169:Irritation from topical therapy:
986:Karamfilov T, Wollina U (1998).
657:10.1111/j.1525-1470.2010.01084.x
607:10.1111/j.1365-2133.2006.07675.x
506:"Generalized pustular psoriasis"
66:) is an extremely rare type of
504:Murphy FR, Stolman LP (1979).
60:Generalized pustular psoriasis
33:Generalized pustular psoriasis
1:
1119:10.1016/s0190-9622(08)80478-1
1041:10.1097/BCR.0b013e31818ba0d3
914:10.1016/0190-9622(91)70025-w
715:Bachelez H, Smahi A (2011).
473:List of cutaneous conditions
287:, suggesting a link between
1208:
264:
239:
952:10.1080/09546630701759587
817:10.1016/j.jid.2016.06.618
1005:10.1080/000155598441576
875:10.4103/0378-6323.57743
248:Leo Ritter von Zumbusch
96:Archives of Dermatology
463:psoriatic arthritis".
299:Infantile and juvenile
267:Impetigo herpetiformis
91:and systemically ill.
734:10.1056/NEJMoa1013068
408:Pediatric Dermatology
323:Circinate and annular
305:Pediatric Dermatology
776:10.1038/jid.2015.288
992:Acta Derm. Venereol
853:Hazarika D (2009).
908:(2 Pt 1): 186–94.
331:See Case Report 4
319:See Case Report 3
163:hydroxychloroquine
75:Signs and symptoms
1179:
1178:
1113:(2 Pt 1): 336–7.
811:(11): 2251–2259.
769:(12): 2964–2970.
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192:Solar irradiation
57:
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27:Medical condition
16:(Redirected from
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591:Griffiths CE
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355:Hydroxyurea
295:increases.
135:salicylates
44:Dermatology
998:(3): 220.
868:(6): 638.
484:References
381:Etretinate
376:Adalimumab
345:Etanercept
335:Treatments
285:progestins
265:See also:
240:See also:
186:Pregnancy
159:penicillin
1192:Psoriasis
1167:: L40.1 (
478:Psoriasis
402:Prognosis
391:Acitretin
364:Systemic
223:Diagnosis
175:anthralin
155:trazodone
68:psoriasis
39:Specialty
1186:Category
1171:L40.100)
1092:71849073
1049:18849840
968:24853353
960:18569276
884:19915261
835:27388993
785:26203641
743:21848462
665:20403118
623:45917573
615:17223864
467:See also
171:coal tar
81:pustules
1127:1918477
1084:5132156
1014:9602231
922:2007662
826:5070969
701:8419106
572:4400368
396:Spevigo
360:Dapsone
209:IL-36RN
131:aspirin
127:lithium
125:Drugs:
89:febrile
85:plaques
1125:
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530:507868
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213:CARD14
102:Causes
46:
1088:S2CID
964:S2CID
858:(PDF)
689:Cutis
619:S2CID
255:Cutis
217:AP1S3
1169:ILDS
1123:PMID
1080:PMID
1045:PMID
1010:PMID
956:PMID
918:PMID
880:PMID
831:PMID
781:PMID
739:PMID
697:PMID
661:PMID
611:PMID
568:PMID
526:PMID
350:PUVA
215:and
83:and
1160:ICD
1115:doi
1072:doi
1037:doi
1000:doi
948:doi
910:doi
870:doi
821:PMC
813:doi
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