A case of cytomegalovirus colitis following immunosuppressive treatment for pyoderma gangrenosum

We report a case of pyoderma gangrenosum (PG) complicated by cytomegalovirus (CMV)-induced colitis. A 79-year-old woman with PG was treated with corticosteroid and cyclosporin. She had blood in her stool and advancing anemia during the treatment. A colonoscopic biopsy specimen from the colon revealed typical CMV-infected cells with CMV inclusions confirmed by immunohistochemistry. Furthermore, there were many CMV-antigen-positive leukocytes, suggesting an active CMV infection, which is serious in compromised hosts. Although ulcerative colitis and Crohn's disease are well known as complications of PG, CMV enterocolitis should be considered in the differential diagnosis of enterocolitis in immunocompromised patients.     

Multifocal and refractory pyoderma gangrenosum: Possible role of cocaine abuse

Pyoderma gangrenosum is a rare and severe inflammatory skin condition. There are different variants, including generalised and atypical forms, but the most common presentation is an enlarging ulcer on the lower extremities. Treatment can represent a challenge for physicians and there are no guidelines based on randomised controlled trials. We report an exceptional case of widespread and refractory pyoderma gangrenosum in a middle‐aged woman where cocaine use may have played a role. Treatment with i.v. pulse corticosteroids and cyclosporine was ineffective, and disease control was obtained with oral corticosteroids together with mycophenolic acid, infliximab and abstinence from cocaine consumption. There was a temporal relationship between disease outbreaks and cocaine consumption and improvement after its discontinuation. In the present case such clinical severity without associated pathology and the temporal association with cocaine abuse raises the possibility of cocaine playing an aetiological role as well as accounting for therapy resistance.     

A case of pyoderma gangrenosum on the stump of an amputated right leg

We present here a case of pyoderma gangrenosum (PG) on the stump of an amputated leg. The patient was a 69-year-old woman who had both of her legs amputated due to acute arterial occlusion. An ulcer first appeared nine years later, after which point it continued to fluctuate in size. Complications included regional blood flow disorder at the amputated stump, diabetes, and secondary infection. Despite various therapies, the ulcer exacerbated, and hypoproteinemia, increased CRP, and fever were confirmed. The patient was diagnosed as having PG based on her clinical symptoms and because the ulcer did not respond to various therapies. The ulcer improved significantly in response to administration of 40 mg/day of prednisolone, and complete epithelialization was later achieved. Given the presence of multiple complications, it was extremely difficult to confirm PG. Therefore, it is important for physicians to consider PG as one of the causes of intractable ulcers.     

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。     

Penile pyoderma gangrenosum: Successful treatment with thalidomide

Pyoderma gangrenosum (PG) is a rare ulcerating inflammatory neutrophilic dermatosis. Different clinical manifestations have been described, including ulcerative, pustular and bullous, and vegetative variants. Classic PG usually occurs on the lower extremities (~70% of cases) but can also involve the hands, head, neck, and scrotum. Genital involvement of PG has rarely been reported. Treatment of the genital PG is usually difficult and resistance to conventional therapeutic regimens was frequently observed. The present authors reported a 16‐year‐old male patient who presented with progressive genital ulceration for 3 weeks. He was treated successfully low dose thalidomide (50 mg/d) and minocycline.     

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。     

Use of Becaplermin for nondiabetic ulcers: pyoderma gangrenosum and calciphylaxis

Large difficult to heal ulcers of various etiologies carry a high morbidity and mortality rate. Becaplermin is a recombinant platelet‐derived growth factor approved for treatment of diabetic ulcers. In this two‐case series, we report the use of becaplermin in the treatment of ulcers due to (i) calciphylaxis, an often fatal condition resulting from systemic calcification and thrombosis of vessels and (ii) pyoderma gangrenosum (PG), a neutrophilic dermatosis. We also report that topical collagenase worsened PG ulcers, consistent with pathergy. Becaplermin can be used to help treat ulcers resulting from calciphylaxis and PG. These encouraging results lend support for the utilization of becaplermin in the treatment of nondiabetic chronic ulcers of various etiologies.     

Treatment of severe recalcitrant pyoderma gangrenosum with ustekinumab

An increased expression of interleukin‐23 has been observed in patients with pyoderma gangrenosum, leading to the use of ustekinumab as a therapeutic option. We report the successful use of ustekinumab in three patients with treatment‐resistant pyoderma gangrenosum of varying clinical presentations.     

Treatment of pyoderma gangrenosum with intravenous immunoglobulin

BACKGROUND: Intravenous immunoglobulin (IVIG) is increasingly being used to treat inflammatory and autoimmune disease. OBJECTIVES: To elucidate the efficacy of IVIG as an adjunct treatment for pyoderma gangrenosum (PG). PATIENTS/METHODS: Ten patients with PG were treated with IVIG at Johns Hopkins Department of Dermatology. All patients had severe mutilating and/or refractory disease requiring multi-agent therapy. The charts were reviewed retrospectively. RESULTS: Seven of the ten patients had clearance of PG lesions in the setting of IVIG and six of these patients maintained efficacy with repeated IVIG treatment. Five patients complained of nausea with treatment, and in one case nausea was severe and intractable. One patient developed an immune reaction requiring diphenhydramine and methylprednisolone and another experienced aseptic meningitis. CONCLUSIONS: IVIG may be an effective adjuvant in the treatment of PG and has an acceptable side-effect profile. Randomized, placebo-controlled, double-blinded trials are needed to confirm this hypothesis.     

Superficial granulomatous pyoderma: an idiopathic granulomatous cutaneous ulceration

A 70-year-old male with a superficial granulomatous ulcer is reported. Histopathological findings were the same as those described for superficial granulomatous pyoderma, a recognized variant of classic pyoderma gangrenosum. The differences between pyoderma gangrenosum and its variant superficial granulomatous pyoderma are highlighted.     

Improvement of ulcerative pyoderma gangrenosum with hyperbaric oxygen therapy

Pyoderma gangrenosum (PG) is a rare noninfectious destructive neutrophilic dermatosis of unknown origin affecting the skin and occasionally the subcutaneous fat. In this report, we present the results of intensive hyperbaric oxygen (HBO) therapy in a 62-year-old Greek woman who had been diagnosed with ulcerative PG two years ago, but had been resistant to other therapies.     

坏疽性脓皮病诊疗研究进展

坏疽性脓皮病(PG)是一种罕见的嗜中性粒细胞皮病,由触发炎症级联后的异常中性粒细胞聚集引起的,主要表现为无菌性中性粒细胞浸润和全身性炎症。最新研究表明药物会引起坏疽性脓皮病,但是相关报道较少,临床工作者对其认识不足。临床中PG易被误诊为难愈合性溃疡而进行清创术治疗,导致病变区扩散恶化,因此正确诊断PG对于疾病的治疗和预后十分重要。本文就PG的诊疗进展进行综述。     

T-cell receptor repertoire in pyoderma gangrenosum: evidence for clonal expansions and trafficking

BACKGROUND: The cause of pyoderma gangrenosum (PG) is unknown, but it is likely to be an immune-mediated disease because it is often associated with conditions such as inflammatory bowel disease and rheumatoid arthritis. T cells play an important role in these conditions and have been implicated in the pathogenesis of other skin diseases such as psoriasis. OBJECTIVES: We examined the T-cell receptor repertoire in PG in order to test the hypothesis that if the T cells were responding to antigen, there would be expanded T-cell clones in the skin and the circulation of these patients. PATIENTS AND METHODS: We studied five patients with PG and examined the T-cell receptor repertoire in cells taken from the peripheral blood and from biopsies of the ulcers, using complementarity determining region 3 spectratyping. RESULTS: We were able to demonstrate expanded clones in the peripheral blood lymphocyte population of each patient. Clonal expansions within the skin were found in four of the five patients. Most significantly, expanded clones that were shared between the blood and the skin were revealed in four of the five patients. CONCLUSIONS: These findings imply that T cells play an integral role in the development of PG and suggest that T cells are trafficking to the skin under the influence of an antigenic stimulus.     

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.     

Two cases of pyoderma gangrenosum complicated with nasal septal perforation

We report two patients with pyoderma gangrenosum complicated with nasal septal perforation. An 18-year-old woman and a 65-year-old man had typical lesions of pyoderma gangrenosum on the legs that responded well to oral prednisolone. Both patients complained of mild nasal discharge, and nasal fibroscopy revealed nasal septal perforation. Biopsy of the nasal lesions showed an active inflammatory infiltrate, mainly of neutrophils. Systemic investigations failed to show any pulmonary or renal lesions of Wegener's granulomatosis. Cytoplasmic immunofluorescent pattern antineutrophil cytoplasmic antibody was negative. In both cases, intense neutrophilic infiltration was observed not only in skin lesions but also in nasal lesions, which may indicate that the nasal lesions had a pathogenesis in common with the skin lesions.     

Efficacy and safety of etanercept for postoperative pyoderma gangrenosum after infliximab serum sickness

Non‐peristomal postoperative pyoderma gangrenosum (PPG) is a rare subtype of pyoderma gangrenosum that occurs in the early postoperative period at surgical incisions, most commonly after breast surgery. Early diagnosis and treatment is essential to prevent severe scaring. TNF‐alpha inhibitor infliximab was reported to be efficient in treatment of PPG refractory to systemic corticosteroids. However infliximab can be not well tolerated. We report the first case of etanercept efficacy in post‐plastic breast surgery pyoderma gangrenosum after infliximab serum sickness.     

Treatment of pyoderma gangrenosum with oral Tripterygium wilfordii multiglycoside

Two patients with refractory pyoderma gangrenosum (PG) were treated with oral Tripterygium wilfordii multiglycoside (TWG). TWG is a Chinese medicine extracted from a medicinal herb, Tripterygium wilfordii Hook F, and has potent anti-inflammatory and immunosuppressive effects. The effect of TWG on PG was demonstrated by clinical findings. Improvement of the lesions occurred within two weeks, and the ulcers healed about a month. Mild side effects such as gastrointestinal disturbances were observed in both patients. These side effects were patient-acceptable, and there was no need to stop the treatment. Transient elevation of serum ALT was observed in one patient; the serum ALT returned completely to normal after the discontinuation of TWG. These results suggest that TWG may be an effective alternative for refractory PG and that careful monitoring of liver function during TWG treatment is necessary.