(von Zumbusch) acute generalized pustular psoriasis | |
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Specialty | Dermatology |
Named after | Leo Ritter von Zombusch |
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]
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]
Kogoj's spongiform pustules can be observed via histopathology to confirm acute GPP. [2]
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]
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.
(von Zumbusch) acute generalized pustular psoriasis | |
---|---|
Specialty | Dermatology |
Named after | Leo Ritter von Zombusch |
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]
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]
Kogoj's spongiform pustules can be observed via histopathology to confirm acute GPP. [2]
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]
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.