Cytapheresis for pyoderma gangrenosum associated with inflammatory bowel disease:A review of current status

Pyoderma gangrenosum(PG)is a neutrophilic dermatosis clinically characterized by the presence of painful skin ulcerations with erythematous.As it is frequently associated with inflammatory bowel diseases,including ulcerative colitis,gastroenterologists should be familiar with the disease including therapeutic options.Therefore,we have conducted a review focusing on the cytapheresis for PG in cases of inflammatory bowel diseases.A literature search was conducted to extract studies published in the last 20 years,with information on demographics,clinical symptoms,treatment,and the clinical course from a total of 22 cases reported and our recent case.In most patients,cytapheresis was associated with improvement or resolution of PG after failure of conventional therapeutic options such as corticosteroids,antibiotics,immunosuppressive agents and immunoglobulin.Based on the information summarized,cytapheresis is helpful in the majority of patients with PG refractory to medical treatment associated with inflammatory bowel diseases and could be further studied in a multicenter,randomized trial.

Pyogenic arthritis,pyoderma gangrenosum,and acne syndrome in a Chinese family:A case report and review of literature

BACKGROUND Pyogenic arthritis,pyoderma gangrenosum,and acne(PAPA)syndrome is a rare autosomal dominant genetic disease characterized by severe autoimmune inflammation,caused by mutations in the PSTPIP1 gene.Due to PAPA heterogeneous clinical manifestation,misdiagnosis or delayed diagnoses are difficult to avoid.With the use of whole-exome sequencing,we identified a missense mutation in the PSTPIP1 gene in a Chinese family.To the best of our knowledge,this is the first case of PAPA reported in China.CASE SUMMARY A 9-year-old boy suffered from recurrent aseptic pyogenic arthritis triggered by minor trauma or few obvious predisposing causes for more than 3 years.Pyogenic arthritis occurred every 3-5 mo,affecting his knees,elbows,and ankle joints.Treatments,such as glucocorticoids,antibiotics,even surgeries could alleviate joints pain and swelling to some extent but could not inhibit the recurrence of arthritis.Similar symptoms were present in his younger brother but not in his parents.According to the whole-exome sequencing,a missense mutation in exon 11 of the PSTPIP1 gene(c.748G>C;p.E250Q)was detected in the boy,his young-er brother and his father.Taking into account the similar phenotypic features with PAPA syndrome reported previously,we confirmed a diagnosis of PAPA syndrome for the family.CONCLUSION In this case,a missense mutation(c.748G>C;p.E250Q)in PSTPIP1 gene was identified in a Chinese family with PAPA syndrome.Previous studies emphasize the fact that PAPA syndrome is hard to diagnose just through the clinical manifestations owing to its heterogeneous expression.Genetic testing is an effectual auxiliary diagnostic method,especially in the early stages of pyogenic arthritis.Only if we have a deep understanding and rich experience of this rare disease can we make a prompt diagnosis,develop the best clinical treatment plan,and give good fertility guidance.

Acute Lymphoblastic Leukemia in Childhood Following Pyoderma Gangrenosum

Pyoderma gangrenosum (PG) is a rare but serious ulcerating skin disease that occurs either idiopathically or associated with various systemic diseases and malignant tumors. Although the association of PG with myeloid malignant tumors is well known, their association with lymphoid malignant tumors, especially in children is extremely rare. We present here a case of PG in a 4-year and 4 months female child, diagnosed in the Pediatrics department, which had initially evolved well with oral corticosteroid therapy and a local dermocorticoid. The development was marked 7 months after stopping corticosteroid treatment by the occurrence of acute lymphoblastic leukemia. This case of PG is presented in the intention of increasing awareness of this uncommon condition with may occurring in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions.

Pyoderma gangrenosum affecting the mouth

Pyoderma gangrenosum is a rare ulcerative cutaneous disorder commonly associated with systemic diseases such as inflammatory bowel diseases. This paper reports a case of pyoderma gangrenosum affecting the mouth and the skin associated with ulcerative colitis. A Medline search in English literature from 1961 to 2012 revealed only 11 documented cases of pyoderma gangrenosum with oral involvement. Previous reported cases of oral cavity involvement have shown that the most common sites of manifestation are the tongue, palate, and tonsillar fauces. This case emphasizes the importance of including inflammatory bowel diseases and their manifestations in the differential diagnosis of ulcerative lesions in the oral cavity.

Successful use of adalimumab for treating fistulizing Crohn's disease with pyoderma gangrenosum:Two birds with one stone

Crohn's disease(CD) is a chronic relapsing and remitting autoinflammatory disorder of the gastrointestinal tract that has many intestinal and extraintestinal complications.The purpose of treatment is long-term remission,reduction of complications,and improvement of patients' quality of life.In many cases,this can be quite challenging and it is necessary to have a well thought out management strategy.We present the case of a 38-year-old woman with fistulizing CD that manifested as diffuse abdominal pain and bloody diarrhea accompanied by arthralgia.In addition,there were ulcerative lesions surrounded by cutaneous inflammation and erythema on her extremities,indicative of pyoderma gangrenosum.The patient was treated with high doses of parenteral methylprednisolone without any improvement and was started on adalimumab.A positive response to adalimumab therapy was observed:after 2 mo of therapy,the ulcerative skin lesion healed completely and the enterogastric fistula was closed after 5 mo adalimumab treatment.Adalimumab might be a suitable initial as well as maintenance therapy in patients with complicated CD.

Pyoderma Gangrenosum with Oral Involvement–Case Report and Review of the Literature

Pyoderma gangrenosum (PG) is a rare, noninfectious neutrophilic dermatosis. Clinically, it begins with sterile pustules that rapidly progress into painful ulcers of variable depth and size with undermined violaceous borders. The diagnosis of PG is based on the history of an underlying disease, a typical clinical presentation, histopathology, and exclusion of other diseases. The peak incidence occurs between the ages of 20 to 50 years with women being more often affected than men. There have been very few reports of pyoderma gangrenosum with oral mucosal involvement. Oral lesions in previously reported cases have included ulcers of varying sizes from a few mm to several cm and have been reported to have been found on the tongue, soft and hard palate, buccal mucosa, and gingiva. Some of these oral lesions have been associated with ulcerative colitis, inflammatory bowel disease, and polycythemia rubra vera. A few cases were reported with biopsy findings, the histological picture being nonspecific, showing ulceration, and necrosis with inflam- matory cell infiltrate. A peculiar case of pyoderma gan- grenosum with an oral lesion is presented here, and the differential diagnosis is discussed.

Intestinal Behcet's disease with pyoderma gangrenosum:A case report

我们这里与脓皮病 gangrenosum 报导肠的 Behcet 的疾病的一个很稀罕的案例。被皮肤的过应性的存在和二个主要标准(口头、生殖器的 aphthoses ) 和一个次要的标准(胃肠的表明) 与肠的 Behcet 的疾病诊断的一个 16 岁的女人，被指我们有左的医院在 1989 年 9 月降低腿溃疡和腹的疼痛。结肠镜检查表明了包含全部冒号的骤发大肠炎。她的更低的腿损害是有不规则的边缘和一个破旧悬于边的痛苦的破坏溃疡。基于这些临床并且实验室调查结果，我们作为与加重的肠的 Behcet 的疾病发展的脓皮病 gangrenosum 诊断了她的皮肤的溃疡。尽管她接受了口头的氢化尼松处理一个月，她的皮肤、肠的损害糟糕被控制。因为有腹激怒的加重的腹的症状，我们在 1989 年 11 月执行了全部的结肠切除术。resected 标本与肠的 Behcet 与深溃疡和嗜中性的累积显示出严重发炎的疾病组织学地兼容。随后，脓皮病 gangrenosum 很快改善了。这堂临床的功课可以建议在脓皮病 gangrenosum 和肠的 Behcet 的疾病之间的靠近的关系。

Successful treatment of pyoderma gangrenosum with concomitant immunoglobulin A nephropathy: A case report and review of literature

Pyoderma gangrenosum(PG)is an uncommon ulcerative cutaneous condition of an unknown etiology and is often associated with immune diseases.However,PG rarely shows visceral involvement,especially in the kidney.A 20-year-old female presented with pedal edema and skin ulceration of both lower limbs.The skin lesion began as an erythematous plaque and then became a blister.She also complained of abdominal distension and a decreasing urine volume.Laboratory data showed high proteinuria,hypoalbuminemia and hyperlipidemia.Her skin and kidney were biopsied.The pathological results indicated PG and immunoglobulin A(IgA)nephropathy.The patient was finally cured with prednisolone in combination with cyclosporine A(CsA).

Diagnosis and management of parastomal pyoderma gangrenosum

Parastomal pyoderma gangrenosum(PPG)is an unusual neutrophilic dermatosis characterized by painful,necrotic ulcerations occurring in the area surrounding an abdominal stoma.It typically affects young to middle-aged adults,with a slight female predominance.The underlying etiology for PPG remains enigmatic but aberrant immune response to injury may play a pivotal role.The reported risk factors for the development of PPG include the presence of extra-intestinal manifestations,autoimmune disorders and obesity,along with local factors,such as the presence of parastomal hernia or pressure ulcer.PPG can develop at any time after the stoma construction.The histopathological features of PPG are not pathognomonic and its diagnosis is mainly based on clinical features.The management of PPG often requires a multidisciplinary approach,with a combination of local wound care and systemic medications.

A case of pyoderma gangrenosum responding to high-dose intravenous immunoglobulin therapy

Pyoderma gangrenosum (PG ) 是稀罕 ulcerative 有特殊特征的皮肤的状况，和原因论还不是清楚的。PG 通常包括 ulcerative 大肠炎和 Crohn 与煽动性的肠疾病被联系“ s 疾病。这个条件在 neutrophilic dermatoses 的光谱以内。PG 的特征不是 specifichistopathologically。通常，它被显著 neutrophilicinfiltrates 的存在在真皮描绘。PG 的处理通常要求全身的 corticosteroids 或 otherimmunospressive 药，并且它的功课是长期的并且 relapsing。一些盒子对这些处理抵抗。在另一方面，那些药的长期的用法自然地引起严肃的副作用，和其他的有效、安全的治疗被要求避免与这些药联系的临床的问题。

MAP3K7、MPO基因与中国人群坏疽性脓皮病发病的相关性研究

目的:对中国人群坏疽性脓皮病患者进行基因检测,明确相关突变。方法:收集坏疽性脓皮病患者外周血,提取DNA并进行目标基因二代测序,使用样本库中对照样本全外显子数据进行基因负荷检验,并对可能与坏疽性脓皮病发病相关的突变位点使用Sanger测序验证。结果:共收集36例坏疽性脓皮病患者,在4例患者的5个基因中发现了6个有害性突变位点。包括一个移码突变:NLRP12:c.908delT(p.F303fs)和5个错义突变:MAP3K7:c.815C>G(p.S272C),MPO:c.1484C>T(p.T495I),MPO:c.1705C>T(p.R569W),NOD2:c.2975G>A(p.R992Q),SYK:c.389A>G(p.E130G)。其中MAP3K7的杂合突变和MPO的纯合(复合杂合)突变仅存在于病例组。结论:本研究发现了MAP3K7(P=7.44E-02),MPO(P=7.44E-02)有害性突变可能与中国人群的坏疽性脓皮病相关。

坏疽性脓皮病治疗进展

坏疽性脓皮病(PG)是一种以皮肤出现复发性、疼痛性及坏死性溃疡为特征的嗜中性皮病。目前尚无PG治疗的共识,且治疗困难。既往糖皮质激素为临床上常用的一线治疗药物,免疫抑制剂等其他传统药物可单独或联合糖皮质激素使用,但部分患者仍难以取得满意疗效。近年来生物制剂及小分子靶向药物逐渐应用于该病的治疗并取得了一定的疗效。本文主要对近年来治疗PG的生物制剂、JAK抑制剂等靶向药物的疗效和安全性进行综述,为临床治疗提供参考。

坏疽性脓皮病的发病机制及治疗进展

坏疽性脓皮病是一种伴随罕见的疼痛性皮肤溃疡的嗜中性皮肤病。坏疽性脓皮病的发病机制复杂且尚未完全清楚,但目前研究表明其与中性粒细胞功能障碍、免疫功能失调、遗传易感性等有关。由于缺乏特异性生物标志物,坏疽性脓皮病的诊断难度较大,容易造成误诊,可根据Paracelsus评分诊断。目前坏疽性脓皮病的治疗以糖皮质激素和免疫抑制剂为主,生物制剂对坏疽性脓皮病的治疗具有广阔的应用前景。

海分枝杆菌感染误诊为坏疽性脓皮病报告1例

68岁男性患者无明显诱因出现右腕背处多发无痛性黄豆大小红斑、丘疹,蔓延至上臂1年余,于外院诊断为坏疽性脓皮病等,并给予免疫抑制治疗;3个月前,右上肢红斑及疼痛加剧,伴破溃,某院皮肤损伤组织病理检测提示符合慢性肉芽肿性炎症,宏基因组二代测序(mNGS)检出海分枝杆菌,故确诊为海分枝杆菌感染。本文就该例海分枝杆菌感染病例进行报告,旨在提高临床对海分枝杆菌感染的认识,做到早诊断、早治疗,改善患者预后。

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。

35例坏疽性脓皮病临床特征及治疗方式探讨

目的探讨坏疽性脓皮病(PG)的临床特征及治疗方式。方法回顾性分析2013年5月至2021年5月就诊于广西壮族自治区人民医院皮肤科门诊及病房的35例PG患者的一般资料、诱因、皮疹分布、皮疹形态、误诊情况、并发症及治疗方式。结果男女患者比例为2.9∶1,年龄29~72(41.5±8.5)岁。15例患者起病前有明确诱因,外伤11例,昆虫叮咬3例,注射吸毒1例。35例患者中溃疡型32例(91.43%),大疱型2例(5.71%),脓疱型1例(2.86%)。起病初期最常被误诊的疾病为细菌性软组织感染[32例(91.43%)]。皮损通常为2个及以上。皮损多发于下肢[23例(65.71%)],以小腿为主。合并有系统性疾病8例(22.86%),其中合并炎症性肠病5例(14.29%),多发性关节炎2例(5.71%),白血病1例(2.86%)。无合并症或合并炎症性肠病、多发性关节炎的患者预后较好,合并血液系统疾病的患者预后差。33例PG患者基于皮损面积的大小及合并症情况,给予强的松0.5~1 mg/(kg·d)。在感染稍控制后,28例患者后续加用环孢素2.5~5 mg/(kg·d)。结论PG的临床表现多种多样,起病初期易误诊,需结合临床表现及治疗效果综合判定。中等至大剂量皮质类固醇激素可有效控制PG,激素联合环孢素治疗安全有效。

坏疽性脓皮病合并反向性银屑病一例患者的护理

报告1例罕见的坏疽性脓皮病合并反向性银屑病患者的护理体会.患者病情重、年龄大且基础疾病较多,给护理工作带来了很大挑战.护理内容主要包括皮肤护理(坏疽性脓皮病溃疡面护理、反向性银屑病皮损护理、压疮护理)、疼痛护理、营养支持、心理护理、基础疾病护理.经过精心治疗和综合性护理干预,患者好转出院.

原发性骨髓纤维化并发增殖型坏疽性脓皮病

报告1例原发骨髓纤维化伴增殖型坏疽性脓皮病。患者男,63岁。左颈后反复肿块伴疼痛2年余。皮肤科检查:左颈后可见一紫红色肿块,中央见深在性溃疡,表面见大量脓性分泌物,边缘隆起,压痛阳性,周围见炎性丘疹及丘脓疱疹。皮损病理组织:真皮及毛囊周围可见大量中性粒细胞浸润,局部中性白细胞聚集形成脓肿。骨髓组织病理符合原发性骨髓纤维化。诊断:原发性骨髓纤维化并发增殖型坏疽性脓皮病。予小剂量糖皮质激素治疗后皮损逐渐消退,停药后仍有复发。