Clinicopathologic Study of Generalized Pustular Psoriasis and Acute Generalized Exanthematous Pustulosis

Lim, Jae Young; Jang, Ho Sun; Oh, Chang Keun; Kwon, Kyung Sool; Kim, Moon Bum

Abstract

BACKGROUND: Generalized sterile pustular eruption with fever which occurs in generalized pustular psoriasis (GPP) and acute generalized exanthematous pustulosis(AGEP) present a diagnostic and therapeutic problems. In Korea, there are a few studies of clinical and histopathologic reviews of these diseases, but long term follow-up and comparative clinicopathologic studies of these two diseases are not available.
OBJECTIVE
This study attempts to identify the differences of these two diseases in the aspects of clinical, laboratory, and histopathologic findings.
METHODS
We evaluated the clinical features, laboratory and histopathologic findings in 41 patients with generalized pustular eruption who had visited Pusan National University hospital during the past 20 years and reviewed the literature.
RESULTS
1. The ratio of patients with GPP(n=32) to ones with AGEP(n=9) was 3.6:1. 2. The mean age at diagnosis was 32.9(male) and 28.9(female) years in GPP, and 10.3 years(male) and 62.8 years(female) in AGEP. 3. The number of patients of GPP with previous personal history of psoriasis vulgaris were 15/32(46.9%) and the number of ones with previous family history of psoriasis vulgaris were 2/32(6.3%). 4. There was no patient of AGEP with personal or familial history of psoriasis. 5. The number of patients with recent drug intake history were 4/32(12.9%) in GPP and 9/9(100%) in AGEP. And common drugs suspected to cause AGEP were antibiotics(4 cases) and analgesics(3 cases). 6. Associated systemic symptoms were fever(37.5%), arthralgia(18.8%), and itching(62.5%) in GPP, whereas 66.7%, 33.3%, and 55.6%, respectively in AGEP. 7. The mean duration of pustules was 32.9 days in GPP and 7.2 days in AGEP. 8. Reccurences of generalized pustular eruption were 46.9% in GPP and 0% in AGEP. 9. Laboratory findings revealed leukocytosis(34.4%), elevated erythrocyte sedimentation rate(28.1%), hypoalbuminamia(25.0%), and eosinophilia(6.3%) in GPP, whereas 77.8%, 55.6%, 33.3%, and 71.4%, respectively in AGEP. 10. GPP and AGEP are diseases sharing similar clinical features, but these two diseases show distinctive clinical, laboratory, and histologic features. We suggest that it is important to be aware of these distinctions for avoidance of unnecessary aggressive therapy indicated for GPP.