Study for pathogenesis of generalized pustular psoriasis

泛发性脓疱型银屑病是一种以急性泛发性外展性脓疱病为特征，伴有全身症状，伴有或不伴有慢性银屑病斑块的银屑病。高热和关节症状，CRP和红细胞沉降率升高，白细胞增多和低钙血症。组织学特征为嗜中性粒细胞侵入表皮，伴小越路海绵状脓疱。泛发性脓疱型银屑病的发病机制尚不明确，但感染、妊娠、月经及药物被认为是重要的促发因素。银屑病和泛发性脓疱型银屑病的遗传背景已经建立。我们对患者的HLA-A、B、C等位基因进行了研究，发现其与泛发性脓疱型银屑病有关.因此，我们调查了HLA-A，B，C血清型和DRB 1和DQB 1基因型的15个无关的泛发性脓疱型银屑病患者和22个脓疱病掌跖在日本北海道。掌跖脓疱病表现为手掌和足底无菌性脓疱，无全身性损害。感染也是该病的诱发因素之一。掌跖脓疱病的HLA研究结果无明显差异。泛发性脓疱型银屑病患者HLA-DRB 1 *0803等位基因显著增高。我们的研究结果表明，HLA-DRB 1 * 0803有助于日本泛发性脓疱型银屑病的易感性。

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.