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.

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 affcer 5 mo adalimumab treatment. Adalimumab might be a suitable initial as well as maintenance therapy in patients with complicated CD.

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

We report here a very rare case of intestinal Behcet＇s disease with pyoderma gangrenosum. A 16-year-old woman who was diagnosed with intestinal Behcet＇s disease by the presence of cutaneous pathergy together with two major criteria （oral and genital aphthoses） and one minor criterion （gastrointestinal manifestations）, was referred to our hospital with a left lower leg ulcer and abdominal pain in September 1989. Colonoscopy demonstrated flare-up colitis involving the entire colon. Her lower leg lesion was a painful destructive ulcer with an irregular margin and a ragged overhanging edge. Based on these clinical and laboratory findings, we diagnosed her cutaneous ulcer as pyoderma gangrenosum developing with exacerbated intestinal Behcet＇s disease. Her cutaneous and intestinal lesions were poorly controlled though she received oral prednisolone treatment for a month. Because of aggravated abdominal symptoms with peritoneal irritation, we performed total colectomy in November 1989. The resected specimen was histologically compatible with intestinal Behcet＇ s disease showing severe inflammation with deep ulcerations and neutrophil accumulation. Subsequently, pyoderma gangrenosum rapidly improved. This clinical course may suggest the close relationship between pyoderma gangrenosum and intestinal Behcet＇s disease.

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.

Diagnostic dilemma between intestinal Behet disease and inflammatory bowel disease with pyoderma gangrenosum

TO THE EDITOR I have read with great interest the very recent article rifled ＂Intestinal Behcet＇s disease with pyoderma gangrenosum： A case report＂ of Nakamura T et al that was published in your journal. The authors stated that they presented a very rare case of intestinal Behcet＇s disease with pyoderma gangrenosum in a 16-year old patient. However, I would like to make some important contributions and suggestions to the presented case and have a few questions to ask the authors.

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.

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.

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.

Pyoderma gangrenosum confused with congenital preauricular fistula infection: A case report

BACKGROUND Pyoderma gangrenosum resulting from or associated with congenital preauricular fistula is rarely reported.CASE SUMMARY We report a rare case of pyoderma gangrenosum misdiagnosed as preauricular fistula infection.To our knowledge,this is the first report to describe pyoderma gangrenosum originating from the site of preauricular fistula.The lesion continued expanding even after combined treatment of systemic antibiotics and thorough debridement.Taking into account the possibility of pyoderma gangrenosum,we applied soft care with normal saline and Vaseline gauze dressing.Systemic corticosteroids were not used until intestinal Clostridium difficile was controlled.No local recurrence was noted at the 12-mo follow-up.CONCLUSION This case highlights the necessity of considering rare diseases,such as pyoderma gangrenosum,when the preauricular sinus deteriorates with general management.The treatment strategy is mutually conflicting between pyoderma gangrenosum and infection of the preauricular sinus.

Atypical presentation of pioderma gangrenosum complicating ulcerative colitis: Rapid disappearance with methylprednisolone

Piodermal gangrenosum (PG) is an uncommon ulcerative cutaneous dermatosis associated with a variety of systemic diseases, including inflammatory bowel disease (IBD), arthritis, leukaemia, hepatitis, and primary billiary cirrhosis. Other cutaneous ulceration resembling PG had been described in literature. There has been neither laboratory finding nor histological feature diagnostic of PG, and diagnosis of PG is mainly made based on the exclusion criteria. We present here a patient, with ulcerative colitis (UC) who was referred to the emergency section with a large and rapidly evolving cutaneous ulceration. Laboratory and microbiological investigation associated with histological findings of the ulcer specimen allowed us to exclude autoimmune and systemic diseases as well as immuno-proliferative disorders. An atypical presentation of PG with UC was diagnosed. Pulse boluses of i.v. methyl-prednisolone were started, and after tapering steroids, complete resolution of the skin lesion was achieved in 3 wk. The unusual rapid healing of the skin ulceration with steroid mono-therapy and the atypical cutaneous presentation in this patient as well as the risk of misdiagnosis of PG in the clinical practice were discussed.

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).

Nasal mucosa pyoderma vegetans associated with ulcerative colitis:A case report

BACKGROUND Pyoderma vegetans(PV)is not a common extra-intestinal manifestation of ulcerative colitis(UC),while nasal mucosa PV associated with UC is particularly rare.CASE SUMMARY We report a 28-year-old female with a history of UC and pyoderma gangrenosum who presented with nasal pain.A nasal lesion could be observed in her nose,and histopathological examination was indicative of PV.The patient was treated with oral prednisone(40 mg per day)with good response and became symptomatically free.There was no recurrent attack after 1 year of follow-up.CONCLUSION Inflammatory bowel disease patients presenting with nasal pain should be further investigated to rule out the coexistence of nasal mucosa PV.

Comparison of Clinical Features between Pyoderma Gangrenosum Concomitant by Inflammatory Bowel Disease and Idiopathic Pyoderma Gangrenosum

Background： Pyoderma gangrenosum （PG） is a rare neutrophilic dermatosis that is highly associated with inflammatory bowel disease （IBD）. Certain PG patients with no systemic disorders have been diagnosed with idiopathic PG. This study sought to clarity/the difference between PG with IBD and idiopathic PG based on clinical features, laboratory tests, and medications. Methods： Twelve patients with PG and IBD and 24 patients with idiopathic PG, who were hospitalized in Peking Union Medical College Hospital from 2000 to 2017, were retrospectively categorized into the IBD group and control group, respectively. Data of clinical features, laboratory tests, and medications were collected and compared between the two groups. Results： Both groups were similar with respect to their clinical features. However, the IBD group had an increased occurrence of arthralgia or arthritis （58.3% vs. 12.5%, P = 0.007）, anemia （83.3% and 29.2%, P = 0.004）, and an increased percentage ofantineutrophilic cytoplasmic antibody （ANCA）-positive patients （85.7% and 0.0%, P 〈 0.001）, compared to the control group. Conclusion： PG patients with IBD had increased occurrence rates of arthralgia or arthritis, anemia, and ANCA-positive status compared to idiopathic PG patients.

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

Pyoderma gangrenosum （PG） is a rare ulcerative cutaneous condition with distinctivecharacteristics, and the aetiology is not clear yet. PG is commonly associated with inflammatory bowel disease including ulcerative colitis and Crohn＇s disease. This condition is within the spectrum of the neutrophilic dermatoses. The features of PG are not specific histopathologically. Commonly, it is characterized by the presence of marked neutrophilic infiltrates in the dermis. The treatment of PG usually requires systemic corticosteroids or other immunospressive medications, and its course is chronic and relapsing. Some cases are resistant to these treatments. On the other hand, long-term usage of those medications naturally causes serious side effects, and an alternative effective and safe therapy is required to avoid the clinical problems associated with the drugs.

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.

Disseminated pyoderma gangrenosum with oral involvement:successful treatment with systemic steroids and cyclosporine

Introduction Pyoderma gangrenosum (PG) is a chronic skin disease characterized by progressive lesions that present as painful suppurative ulcers and tend to recur. The pathogenesis of the disease is incompletely under-stood;however, 50%to 70%of affected persons have an associated concomitant systemic disease such as inflammatory bowel disease (IBD) (1)Any site of the body can be affected;however, involvement of the oropharynx is rare. Here, we present a patient with disseminated PG with oropharyngeal involvement.

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。

依奇珠单抗治疗坏疽性脓皮病一例并文献复习

坏疽性脓皮病是一种少见的嗜中性皮肤病,其发病机制目前尚不完全清楚,传统治疗包括系统应用糖皮质激素及环孢素、环磷酰胺等免疫抑制剂,但常规药物疗效并不十分令人满意。近年来,随着生物制剂在皮肤科领域的广泛应用,国外有越来越多超适应症应用生物制剂治疗坏疽性脓皮病的经验报道。现报道IL-17A抑制剂依奇珠单抗治疗坏疽性脓皮病一例,并对既往文献进行复习。

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。

阿达木单抗联合糖皮质激素治疗复发难治性坏疽性脓皮病一例

本文报道一例患有坏疽性脓皮病17年的患者,该患者先后18次于我科住院治疗,曾使用糖皮质激素、免疫抑制剂、益赛普、沙利度胺等药物及物理治疗,皮肤溃疡可好转,但仍有反复。半月前患者病情加重,给予糖皮质激素联合阿达木单抗治疗,2周后溃疡变浅出院。出院后口服甲泼尼龙50 mg每日1次,逐渐减量,阿达木单抗40 mg,每2周1次,第4次注射阿达木单抗后溃疡大部分愈合。随访8个月,患者已停用糖皮质激素,阿达木维持治疗,皮损基本痊愈。