Pyoderma gangrenosum on the breast: A case presentation and review of the published work

We present a case of pyoderma gangrenosum localized on the breast, without a preceding surgical intervention and associated systemic disorder. The ulcer had rapidly developed and covered a large portion of the breast. The patient responded well to systemic steroids and salicylazosulfapyridine and the ulcer completely healed with scarring after 3 months of treatment. Pyoderma gangrenosum rarely involves the breasts. A published work survey disclosed only 31 reported cases up to date. In most of these cases the lesions were related to previous surgical interventions, probably as the result of a pathergy phenomenon. The main differential diagnoses were skin and soft tissue infections including necrotizing fasciitis, and malignant neoplasms. Negative initial wound cultures and the relative sparing of nipple/areola complex helped to eliminate these disorders. Though an unusual site for pyoderma gangrenosum, lesions on the breast showed the characteristic clinical features of the disease. The types of associated disorders were also similar to those of the cases with classical pyoderma gangrenosum. As most of the lesions healed with significant scarring, early recognition and treatment of pyoderma gangrenosum located on the breast is important to prevent serious physical and psychological morbidity.     

Sporotrichosis masquerading as pyoderma gangrenosum: case report and review of 19 cases of sporotrichosis

We present the case of a 59-year-old woman who had large ulcerations on her right leg that were diagnosed initially as pyoderma gangrenosum and treated with three immunosuppressive agents (cyclosporin, prednisone and azathioprine) for 6 months. Results of a biopsy at 6 months showed numerous cigar-shaped bodies consistent with Sporothrix schenckii; identification was confirmed by tissue culture. A retrospective review was performed for all cases diagnosed as sporotrichosis from tissue culture or biopsy specimens at the Mayo Clinic. Nineteen cases were identified. The present case was the only one in which fungal organisms were visible on histological examination. The present case emphasizes the importance of making a definitive histological diagnosis in unusual ulcer cases or in suspected cases of pyoderma gangrenosum before the initiation of immunosuppressive therapy. The large number of cigar-shaped bodies in the tissue is a rare finding in sporothrix infection and has been reported in only two cases previously.     

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。     

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.     

Bullous pyoderma gangrenosum of the bilateral dorsal hands

Pyoderma gangrenosum (PG) shows characteristic non‐infectious ulcers that are commonly associated with systemic diseases such as inflammatory bowel diseases, myeloproliferative disorders or aortitis syndrome. The typical clinical appearance is undermining ulcers with reddish and irregular borders on the legs. As PG has these notable signs, the diagnosis is relatively easy and its treatment depends on the severity of underlying complications. We report a case of a 60‐year‐old Japanese man, diagnosed with bullous PG, who also had been suffering from myeloperoxidase antineutrophil cytoplasmic antibody‐positive microscopic polyangiitis and pulmonary aspergillosis. This case displayed soft whitish ulcers that existed on the rough ulcer base, with irregular borders, on his bilateral dorsal hands. Initially, it seemed to be cutaneous secondary aspergillosis because the host was already infected with pulmonary aspergillosis in both lungs. The differential diagnosis of PG from aspergillosis was from the sterile bullae or neutrophilic bullae on his right forearm, which evolved into ulcers in a few days. This case was finally diagnosed as bullous PG and a topical glucocorticoid was very effective to epithelize the ulcers in 2–3 weeks.     

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.     

Pediatric pyoderma gangrenosum associated with leukocyte adhesion deficiency type 1: A case report and review of the literature

Pyoderma gangrenosum is a rare neutrophilic dermatosis characterized by painful skin ulcers with necrotic, undermined margins. In severe cases, particularly in pediatric patients, work-up for an associated autoimmune, inflammatory, malignant, or genetic disorder should be considered based on the clinical presentation. We report a unique case of pediatric pyoderma gangrenosum with a leukemoid reaction, secondary to an autosomal recessive leukocyte adhesion deficiency type 1.     

Pyoderma gangrenosum successfully treated with golimumab: Case report and review of the literature

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis frequently related to chronic inflammatory bowel disease (IBD) often associated with exacerbation of intestinal disease and/or loss of treatment efficacy. However, in patients with comorbidities, such as diabetes, the diagnosis may be a challenge. Here, we report the case of a 68‐year‐old man with a history of ulcerative rectocolitis (URC), type II diabetes and arterial hypertension, who had been treated with infliximab and adalimumab in the past. In September 2017, patient developed an erythematous, infiltrated and painful lesion of the third distal part of his left leg, with ulcerative evolution, rapidly worsened despite a broad‐spectrum antibiotic treatment had been introducted. A worsening of rectocolitis occurred simultaneously. In agreement with the gastroenterologists, patient started a new biological therapy with golimumab, and oral prednisone with slow tapering of steroid dosage following the improvement of both cutaneous and intestinal symptoms. Dermatologists should be aware about the risk of PG in patient suffering from IBDs, and consider this diagnosis in all patients affected by URC developing rapidly extending ulcerative skin lesion. Moreover, therapeutic choice should take into consideration the effectiveness of golimumab on the inflammatory background, which sustains both intestinal and skin disease in this type of patients.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

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。     

THERAPEUTIC HOTLINE: Infliximab for treatment of resistant pyoderma gangrenosum associated with ulcerative colitis and psoriasis. A case report

Pyoderma gangrenosum (PG) is a rare, noninfectious, neutrophilic dermatosis of unknown origin that is associated with systemic diseases in 50% of cases. The authors present a case of a 54‐year‐old man patient with refractory to conventional treatment PG associated with ulcerative colitis and psoriasis, which showed a successful response to treatment with infliximab, a chimeric monoclonal antibody that inhibits tumor necrosis factor alpha (TNF‐α). This case report shows the frequent difficulty in the therapeutic approach of PG, especially if associated with underlying disease, and necessity to apply new agents, such as a novel application of the TNF‐α inhibitors, in relationship to the recent pathogenic knowledge.     

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.     

阿达木单抗治疗坏疽性脓皮病一例并文献复习

报道阿达木单抗成功治疗一例坏疽性脓皮病,并进行文献复习。患者,男,53岁。左股内侧红斑、丘疹6个月,溃疡3个月。组织病理示：符合坏疽性脓皮病。给予雷公藤多苷片、沙利度胺片治疗3天,仍有新发红斑、丘疹,于第3天、第10天分别给予皮下注射阿达木单抗80 mg、40 mg,后每2周皮下注射阿达木单抗40 mg,注射第4剂阿达木单抗时溃疡已愈合。半年后随访,皮损未复发。     

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.     

Successful treatment of classic pyoderma gangrenosum with intralesional infliximab injection: A case report

Pyoderma gangrenosum (PG) is a rare dermatological disease characterized by the rapid development of painful skin ulcers. While systemic infliximab is considered a standard treatment for patients with PG, herein, we report our success with the use of intralesional infliximab in a 40-year-old woman with systemic lupus erythematosus (SLE) and PG.     

坏疽性脓皮病合并类白血病反应1例

患者男,17岁,双下肢反复疼痛溃疡3年。本次复发前无明显诱因出现高热,双胫前出现丘脓疱疹,疼痛,范围迅速扩大,并破溃形成溃疡。结合临床表现及组织病理改变,诊断为坏疽性脓皮病。外周血显示白细胞高达114×109/L,血小板低至1.9×109/L,同时伴有贫血,经骨髓涂片、中性粒细胞碱性磷酸酶积分以及对治疗的反应,证实该患者为坏疽性脓皮病并发类白血病反应。