副肿瘤性天疱疮并发局灶性Castleman病一例

A case of paraneoplastic pemphigus associated with localized Castleman disease

27岁女性患者,口腔白斑、水疱、溃疡伴疼痛3个月余,外生殖器糜烂、溃疡伴瘙痒、疼痛2个月.皮肤科情况:口腔黏膜及舌部可见散在白斑、水疱、糜烂及溃疡;口唇可见糜烂、溃疡和血痂;双眼周、鼻腔内可见糜烂、血痂,上覆分泌物;外生殖器可见多个糜烂及溃疡面,上覆黄绿色分泌物;双手、足可见角化性紫红斑;头皮脱发区拉发实验阳性.皮损组织病理示表皮基底层上方松解、大疱形成,局灶性真皮乳头水肿,伴淋巴细胞等炎性细胞浸润.胸部X线检查提示右下后纵隔占位性病变.胸膜穿刺活检:纤维组织增生,伴较多形态较成熟的淋巴细胞浸润.免疫组化染色:CD20、CD79a、SMA、CD34、CD21、Calponin、CD3、Ki67、CD38均阳性.诊断:副肿瘤性天疱疮合并局灶性Castleman病.给予甲泼尼龙和沙利度胺治疗病情无明显好转,转外科行手术治疗,患者因未及时治疗去世.

A 27-year-old female patient presented with oral leukoplakia,blisters and ulcers with pain for more than 3 months,external genital erosion and ulcers with pruritus and pain for 2 months.Physical examination revealed leukoplakia,blisters,erosion and ulcers on oral mucosa and tongue;erosion,ulcers,blood crusts and exudation involved the periocular skin,lips,nasal cavity and external genitalia;keratotic purplish erythema on both hands and feet;hair pull test was positive in the hair loss area of the scalp.The histopathological examination showed that acantholysis above the basal layer of epidermis,blisters developed,and focal papillary dermal edema accompanied by infiltration of lymphocytes and other kinds of inflammatory cells.Both chest X-ray and chest CT showed space occupying lesions in the right lower posterior mediastinum.CT-guided pleural puncture biopsy suggested that fibrous tissue hyperplasia with more mature lymphocytes infiltration.Immunohistochemical staining:CD20,CD79a,SMA,CD34,CD21,calponin,CD3,Ki67 and CD38 were positive,so giant lymph node hyperplasia(Castleman disease)and hyaline vascular type were considered.To sum up the diagnosis was paraneoplastic pemphigus complicated with focal Castleman disease.The patient was treated with methylprednisolone and thalidomide,but the condition did not improve significantly,and the patient was transferred to surgery for surgical treatment,then passed away due to failure to receive timely treatment.