Leukocytapheresis treatment for pyoderma gangrenosum

A 42-year-old man presented with painful erythema with pustules and multiple small ulcers on the shins. He had suffered from ulcerative colitis (UC) and received oral glucocorticosteroid and salicylazosulfapyridine therapies for 7 years. Biopsy of the lesion demonstrated mixed cellular infiltrates with dominant neutrophils. The patient was diagnosed with pyoderma gangrenosum (PG) and underwent leukocytapheresis (LCAP), an extracorporeal leucocyte removal therapy, once a week for 5 weeks without changing the doses of the oral medications. The skin lesions as well as clinical signs of UC rapidly improved after LCAP, and no recurrence was seen during a follow-up period. There were no major complications during LCAP. LCAP will provide an effective and safe tool for the treatment of PG.     

浅表性大疱型坏疽性脓皮病

报告1例浅表性大疱型坏疽性脓皮病。患者女，58岁。四肢红斑、水疱、溃疡伴疼痛9d入院。组织病理表现为表皮内有一大脓疱，脓疱两侧表皮内有较多中性粒细胞侵入，两侧表皮有明显细胞间及细胞内水肿，真皮浅层及中层有弥漫性中性粒细胞浸润，有明显核尘，核碎裂，真皮胶原纤维间水肿，皮下组织有部分区域出血。类似于典型坏疽性脓皮病，但是在真皮中的位置更表浅。给予小剂量糖皮质激素，米诺环素，雷公藤多苷治疗，2个月后痊愈。     

Pyoderma gangrenosum with liver, spleen and bone involvement in a patient with chronic myelomonocytic leukaemia

Pyoderma gangrenosum is a neutrophilic dermatosis of unknown aetiology. Visceral involvement by pyoderma gangrenosum is rare, the lung being the most frequent site of extracutaneous disease. We describe a 73-year-old man with pyoderma gangrenosum and chronic myelomonocytic leukaemia in whom aseptic hepatosplenic abscesses and bony lesions were associated.     

Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra

The triad of sterile pyogenic arthritis, pyoderma gangrenosum and acne is known by the acronym of PAPA syndrome. It is a rare autosomal dominant disease of early onset. The treatment of pyoderma gangrenosum is challenging as there is often only partial response to systemic glucocorticosteroids and immunosuppressive therapies. We report the rapid and lasting response of pyoderma gangrenosum to the targeted treatment with the recombinant human interleukin-1 receptor antagonist (rHuIL-1Ra) anakinra in a patient with PAPA syndrome.     

坏疽性脓皮病19例临床分析

目的:提高对坏疽性脓皮病(PG)的认识,探讨其最有效的治疗方法。方法:对1994—2004年在我科住院的19例PG患者的临床资料进行回顾性分析。结果:该病发病年龄以45岁以上居多;PG除有皮肤损害外,还可累及其他器官。约42.1%的患者并发有系统性疾病,其中以并发糖尿病居多。实验室检查结果和组织病理改变无特异性。结论:PG是一种少见的皮肤病,主要根据其临床特点诊断。目前,糖皮质激素是控制PG最有效的药物,亦可联合应用免疫抑制剂、磺胺类、米诺环素等药物,局部治疗有利于皮损愈合。     

坏疽性脓皮病12例临床分析

报告12例坏疽性脓皮病患者的性别与年龄构成、并发症、实验室检查、治疗经过和结果,对该病的病因、治疗和预后进行了讨论。     

Persistent facial ulcerations with a unique clinical appearance: possible trigeminal trophic syndrome

We reported a 53-year-old woman with a persistent facial ulcer which moved gradually from the right marginal portion of the nasal root to the right lateral canthus along the margin of the right lower eyelid. The ulcer healed spontaneously. A similar ulcer developed at the entrance of the right anterior naris. The clinical findings were suggestive of squamous cell carcinoma, deep mycosis, mycobacterial infection, or a related condition. However, no definitive diagnosis could be made from the clinicopathological findings or the laboratory examinations, including bacterial, mycobacterial, and fungal cultures. The characteristic location of the lesion, and course of the disease, however, suggested the diagnosis of trigeminal trophic syndrome.