Pyoderma gangrenosum in association with juvenile rheumatoid arthritis

A 17-year-old girl presented with multiple, painful, erythematous blisters and ulcerated lesions on the shins and buttocks. She also had arthralgia. She had suffered from juvenile rheumatoid arthritis (JRA) and received anti-inflammatory agents and oral glucocorticoids for eight years. A biopsy of a lesion showed epidermal ulceration with marked neutrophilic infiltrates in the dermis. The patient was diagnosed with pyoderma gangrenosum (PG). PG is an uncommon cutaneous ulceration within the spectrum of the neutrophilic dermatoses that is reported in association with a number of systemic disorders, including inflammatory bowel disease, hematologic disease, internal malignancies, arthritis, immune abnormalities, and solid tumors. To our knowledge, this is the first reported case of PG associated with JRA.     

Pyoderma gangrenosum

ABSTRACT: Pyoderma gangrenosum (PG) is a chronic ulcerative skin disease with striking clinical features that may rarely involve internal organs, particularly the lung, liver, and spleen. Approximately 50% of PG patients have associated inflammatory, immune dysfunction, or neoplastic diseases. The various topical, systemic, and surgical treatment options for PG are reviewed here. Systemic corticosteroids plus immunosuppressive therapy are often required to successfully manage severe cases of PG. Practical treatment strategies are discussed along with their proper monitoring and potential adverse effects. Current treatment methods are empiric, and the optimum treatment regimen for this disorder requires future controlled clinical trials.     

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。     

坏疽性脓皮病合并炎症性肠病

报告1例坏疽性脓皮病合并炎症性肠病。患者男,30岁。腹痛、腹泻及稀薄脓血便1个月余;躯干及四肢出现散在的红色丘疹、脓疱疹,溃疡伴疼痛12 d入院。皮损组织病理检查:表皮角化过度,棘层增厚,真皮全层及皮下脂肪间大量中性粒细胞浸润,可见红细胞外渗及血管壁纤维蛋白样变性。电子肠镜诊断为炎症性肠病。抗核周型中性粒细胞胞质抗体(anti perinuclear antineutrophil cytoplasmic antibody,p-ANCA)阳性。予糖皮质激素、美沙拉嗪及支持治疗后,患者病情明显好转。     

Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin

A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.     

Pyoderma gangrenosum as first clinical manifestation of gastric adenocarcinoma

Pyoderma gangrenosum (PG) is a neutrophilic dermatosis of unknown etiology characterized by typical skin ulcers. It may be related to systemic disorders but its association with solid tumors is very unusual. In this setting, we describe a patient in whom PG was the first and isolated manifestation of advanced gastric adenocarcinoma.     

Pyoderma gangrenosum associated with severe oropharyngeal involvement and IgA paraproteinaemia

We report a patient with combined cutaneous and oropharyngeal pyoderma gangrenosum in association with an IgA lambda paraproteinaemia. The differential diagnosis of oral pyoderma gangrenosum is discussed.     

Pyoderma gangrenosum with spleen involvement and monoclonal IgA gammopathy

A 46-year-old man with a 3-year history of pyoderma gangrenosum was admitted with ulceration (6 x 5 cm), on the right leg. Previously he had been treated with tapering doses of prednisone (maximum dose 1 mg/kg per day); however, he had had a few exacerbations following each taper of prednisone dose. Immunoelectrophoresis demonstrated monoclonal IgA gammopathy of lambda light chains. Abdominal echography and abdominal computed tomographic scan revealed multiple splenic abscesses. Treatment was started with oral prednisone (1 mg/kg per day) and cyclosporin (5 mg/kg per day) and 6 weeks later complete remission was achieved. Systemic involvement in pyoderma gangrenosum is very rare, and according to our knowledge there are only a few cases with spleen involvement.     

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。     

Pyoderma gangrenosum or leukaemia cutis?

We report a case of atypical bullous pyoderma gangrenosum associated with acute myeloid leukaemia in which we found atypical myeloid cells within the skin lesion. Although there have been many reported cases of leukaemia-associated pyoderma gangrenosum, the finding of myeloblasts in the skin has rarely been described.     

Pyoderma gangrenosum in a patient with autoimmune haemolytic anaemia and complement deficiency

About 50% of cases of pyoderma gangrenosum (PG) are associated with a variety of systemic diseases. We describe a patient with severe PG on both legs who suffered from an autoimmune haemolytic anaemia in association with a congenital deficiency of complement factors C2 and C4. To the best of our knowledge, this constellation has not been previously reported.     

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.     

Pyoderma gangrenosum requiring inpatient management: a report of 26 cases with follow up

We present a case series of inpatients with pyoderma gangrenosum (PG), an ulcerative neutrophilic skin condition of unknown aetiology. Twenty-six patients were admitted with PG, nine men and 17 women. At the time of the chart review, seven patients (26.9%) had died. Patients had a mean of 2.0 active ulcerative lesions and 22 patients' ulcers (84.6%) were on the lower limb. Systemic diseases were coexistent in 15 patients (57.7%), the most common being rheumatoid arthritis (19.2%). Thirty-eight wound cultures were taken and were positive for Staphylococcus aureus in 22 cases (57.8%) and Pseudomonas aeruginosa in 20 (52.6%). After prednisolone, cyclosporin was the next most commonly prescribed systemic therapy (34.6%). Surgical debridement was undertaken in seven cases (26.9%) and two patients had skin grafts. Upon discharge from hospital, 21 patients' ulcers (80.8%) had improved. At 6 months 50% showed complete ulcer healing. Our results highlight the potential severity of PG requiring hospital admission, the need for aggressive therapy and the overall high associated morbidity and mortality.     

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.     

Pyoderma gangrenosum with increased levels of serum cytokines

A 66‐year‐old woman presented after an episode of accidental trauma with a painful ulcer on her scalp which rapidly enlarged in size, accompanied by central necrosis and undermining ulceration. The patient's past history was negative for underlying systemic disease, although she had had a similar post‐traumatic intractable leg ulcer 3 years prior, which was unresponsive to surgical management but successfully treated with systemic steroids. A biopsied specimen from the scalp showed marked neutrophilic infiltrates in the dermis, compatible with a diagnosis of pyoderma gangrenosum (PG). The large ulcerative lesion responded very well to oral steroid therapy, showing rapid epithelialization. Serum levels of granulocyte colony‐stimulating factor and interleukin‐6 were significantly elevated prior to treatment, with decrease to normal levels after treatment. Serum tumor necrosis factor (TNF)‐α and granulocyte macrophage colony‐stimulating factor levels were within normal limits. The significance and pathogenic role of cytokine burst in PG is reviewed and discussed.     

坏疽性脓皮病合并无菌性肺脓肿及类风湿性关节炎1例

报告1例坏疽性脓皮病合并无菌性肺脓肿及类风湿性关节炎。患者女，65岁。小腿反复破溃2年，加重20d人院。既往有类风湿性关节炎10余年。入院查胸部CT示双肺多发性结节空洞．肺结节穿刺抽出灰黄色脓性液体，脓液未查到病原菌。给予皮质类固醇治疗，皮损及肺部病变均有好转。     

Pyoderma gangrenosum associated with nasal septal perforation, oropharyngeal ulcers and IgA paraproteinemia

We report a case of pyoderma gangrenosum (PG) associated with nasal septal perforation, pharyngeal ulcers and IgA paraproteinemia. A 28-year-old woman first developed painful undermined ulcers on her perianal, inguinal and axillary areas when she was 22 years old. Histological findings from the cutaneous ulcers showed dermal and epidermal infiltrate of neutrophils, which was compatible with PG. Laboratory examinations did not detect any associations of systemic diseases other than polyclonal IgA paraproteinemia. Nasal fiberscopy revealed septal perforation and multiple ulcers on her pharynx. The biopsy specimen from the pharyngeal ulcers showed a polymorphous cellular infiltrate without necrotizing vasculitis or granuloma. However, there were no atypical lymphocytes that are typically seen in nasal NK/T lymphoma. By immunohistochemical analysis, the infiltrated lymphocytes were proved to be T cells and Epstein-Barr virus encoded RNA (EBER) was not detected. No pulmonary or renal lesions resembling Wegener's granulomatosis were found. Taken together, the nasal septal perforation was considered as nasal involvement of PG.