Two cases of generalized pustular psoriasis complicated by IgG4-related disease.
Two cases of generalized pustular psoriasis complicated by IgG4-related disease.
复制标题
全身性脓疱型银屑病并发 IgG4 相关疾病 2 例。
DOI：
10.1111/bjd.16712
复制
发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Shimizu H.
中科院分区：
文献类型：
--
作者：
Miyazawa H;Fujita Y;Iwata H;Ishikawa Y;Nishio S;Ishijima K;Shinmei Y;Takeichi T;Goto K;Oi R;Akiyama M;Shimizu H.
亲爱的编辑，IgG4相关疾病（IgG4-RD）的特征是IgG4阳性浆细胞在各种器官中的浸润和血清IgG4的升高。IgG4-RD表现出各种皮肤变化，包括银屑病样皮疹。1然而，迄今为止，尚未报道全身性脓疱性银屑病（GPP）并发IgG4-RD。我们提出两个这样的情况下，在案件1，68岁的日本妇女谁被诊断为GPP在1998年被转介给我们后，复发GPP在2002年。体格检查显示躯干上有多个新鲜的红色丘疹和丘疹混合脓疱（图1a），其组织病理学特征为Kogoj海绵状脓疱。GPP发作时，患者眼睑和颌下腺肿胀（图1b）。磁共振成像显示泪腺对称性肿胀（图1c），从而诊断为米库利奇病（MD）。MD对每日30 mg口服泼尼松龙（PSL）有反应（图1d）。此后，她出现高丙种球蛋白血症性紫癜和膜性肾病（MN）。她目前在口服10 - 20 mg/天的依维替酸和75 mg/天的环孢素（CsA）治疗GPP和5 mg/天的PSL治疗MD和MN后没有不良症状。后来，血清IgG 4随后被证明升高（215 mg dL/dl，正常范围4 - 108 mg dL/dl）。
DEAR EDITOR, IgG4-related disease (IgG4-RD) is characterized by infiltration of IgG4-positive plasma cells in various organs and elevation of serum IgG4. IgG4-RD manifests various skin changes including psoriasis-like rashes. 1 However, generalized pustular psoriasis (GPP) complicated by IgG4-RD has not been reported to date. We present two such cases below.In case 1, a 68-year-old Japanese woman who had been diagnosed with GPP in 1998 was referred to us following a recurrence of GPP in 2002. Physical examination revealed multiple fresh red erythemas and papules mixed with pustules on the trunk (Fig. 1a), which were histopathologically characterized by spongiform pustules of Kogoj. At the time of GPP onset, the patient had swelling of the eyelids and submandibular glands (Fig. 1b). Magnetic resonance imaging showed symmetrical swelling of the lacrimal glands (Fig. 1c), which led to the diagnosis of Mikulicz disease (MD). MD responded to oral prednisolone (PSL) at 30 mg daily (Fig. 1d). Thereafter, she developed hypergammaglobulinaemic purpura and membranous nephropathy (MN). She is currently free from adverse symptoms following the oral administration of etretinate at 10–20 mg daily and ciclosporin (CsA) at 75 mg daily for GPP, and PSL at 5 mg daily for MD and MN. Later, serum IgG4 was subsequently proven to be elevated (215 mg dL À1, normal range 4–108 mg dL À1).