Study for pathogenesis of generalized pustular psoriasis

全身性脓疱性银屑病的特点是急性全身性发疹性脓疱病，有或没有慢性银屑病斑块。注意到高热和关节症状，CRP升高和红细胞沉降率，白细胞增多和低钙。中性粒细胞侵入表皮并伴有海绵状脓疱是其组织学特征。全身性脓疱性银屑病发病机制尚不明确，但感染、妊娠、月经和药物被认为是重要的诱发因素。银屑病和广泛性脓疱性银屑病的遗传背景已经确定。我们研究了患者的HLA- A，B， C等位基因，发现与广泛性脓疱性银屑病有关。为此，我们对日本北海道地区15例广泛性脓疱性银屑病患者和22例掌跖脓疱性银屑病患者的HLA-A、B、C血清型和DRB1、DQB1基因型进行了研究。掌跖脓疱病是指手掌和脚底的无菌脓疱，具有全身症状。感染也是这种疾病的诱发因素之一。掌跖脓疱病的HLA无明显偏差。HLA-DRB1*0803等位基因在全身性脓疱性银屑病患者中显著升高。结果提示HLA-DRB 1 * 0803与日本广泛性脓疱性银屑病的易感性有关。

Generalized pustular psoriasis is characterized by acute generalized exanthematous pustulosis with general sympstomes in whom do or doesn't has chronic psoriatic plaques. High fever and joint symptome, elevated CRP and erythrocyte sedimentation rate, leukocytosis and hypocalcemiaare noted. Histological features in which neutrophilic invasion into the epidermis with Kogoji's spongiform pustules are characteristic. The pathogenesis of generalized pustular psoriasis remains uncertain, however, infection, pregnancy, menstruation and drugs are considered to be important precipitating factors. Genetic background for psoriasis and generalized pustullar psoriasis has founded. We had studied HLA- A,B, C allels of the patients and found an association to generalized pustular psoriasis. Thus, we investigated the HLA-A,B,C serotypes and DRB1 and DQB1 geno types of 15 unrelated patients with generalized pustular psoriasis and 22 pustulosis palmoplantaris in Hokkaido, Japan. Pustulosis palmoplantaris represents aseptic pustules on palms and soles withoy systemic symptomes. Infections are one of the precipitaing factors of this diseas as well. Pustulosis palmoplantaris showed no significant deviation in HLA studied. The HLA-DRB1*0803 allele was significantly increased in the patients with generalized pustular psoriasis. Our results suggest that HLA-DRB 1 * 0803 contribute to the susceptibility to the Japanese generalized pusutular psoriasis.