BACKGROUND Pyoderma gangrenosum(PG)is one of the most severe extra-intestinal manifest-ations of ulcerative colitis(UC).The treatment of refractory UC combined with PG is challenging,particularly for patients with sch...BACKGROUND Pyoderma gangrenosum(PG)is one of the most severe extra-intestinal manifest-ations of ulcerative colitis(UC).The treatment of refractory UC combined with PG is challenging,particularly for patients with schizophrenia(SCZ)with a long-term history of risperidone use,and there have been no successfully treated patients reported in the literature.CASE SUMMARY A 36-year-old woman attended the gastroenterological clinic due to intermittent symptoms of diarrhea and mucous bloody stools.Prior to the emergence of these symptoms,the patient had a history of SCZ for 3 years.She had been receiving long-term risperidone treatment and had stable mental symptoms.In April 2023,she was diagnosed with UC E3 moderate and began taking mesalazine 3 g/day.In March 2024,her intestinal symptoms recurred and approximately 2 months later,PG developed in both lower limbs.Previous treatments with adalimumab and steroids were ineffective for PG and UC,and simultaneously,the patient experienced headache,confusion,and severe sleep disturbances.After switching to upadacitinib(UPA)45 mg/day,PG lesions showed complete healing and fecal calprotectin was<10μg/g after 7 weeks of treatment.Following approximately 12 weeks of UPA therapy,colonoscopy indicated that the patient had achieved mucosal healing.No adverse events occurred during UPA induction and main-tenance therapy for 6 months with risperidone.CONCLUSION UPA treatment led to successful resolution of both intestinal and extra-intestinal manifestations in this patient with new-onset UC who had a history of SCZ.No adverse effects were observed with concurrent UPA and risperidone use.展开更多
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...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.展开更多
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 re...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.展开更多
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 tw...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.展开更多
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 autho...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.展开更多
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 ulc...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.展开更多
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 PAP...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.展开更多
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 ...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</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> 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.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ur</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ring</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions</span></span></span><span style="font-family:Verdana;">.展开更多
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 th...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.展开更多
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...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).展开更多
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 idi...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.展开更多
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 ...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.展开更多
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 ad...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.展开更多
文摘BACKGROUND Pyoderma gangrenosum(PG)is one of the most severe extra-intestinal manifest-ations of ulcerative colitis(UC).The treatment of refractory UC combined with PG is challenging,particularly for patients with schizophrenia(SCZ)with a long-term history of risperidone use,and there have been no successfully treated patients reported in the literature.CASE SUMMARY A 36-year-old woman attended the gastroenterological clinic due to intermittent symptoms of diarrhea and mucous bloody stools.Prior to the emergence of these symptoms,the patient had a history of SCZ for 3 years.She had been receiving long-term risperidone treatment and had stable mental symptoms.In April 2023,she was diagnosed with UC E3 moderate and began taking mesalazine 3 g/day.In March 2024,her intestinal symptoms recurred and approximately 2 months later,PG developed in both lower limbs.Previous treatments with adalimumab and steroids were ineffective for PG and UC,and simultaneously,the patient experienced headache,confusion,and severe sleep disturbances.After switching to upadacitinib(UPA)45 mg/day,PG lesions showed complete healing and fecal calprotectin was<10μg/g after 7 weeks of treatment.Following approximately 12 weeks of UPA therapy,colonoscopy indicated that the patient had achieved mucosal healing.No adverse events occurred during UPA induction and main-tenance therapy for 6 months with risperidone.CONCLUSION UPA treatment led to successful resolution of both intestinal and extra-intestinal manifestations in this patient with new-onset UC who had a history of SCZ.No adverse effects were observed with concurrent UPA and risperidone use.
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
基金Supported by the National Natural Science Foundation of China,No.81770875the Post-Doctor Research Project,West China Hospital,Sichuan University,No.19HXBH053+1 种基金the Health and Family Planning Commission of Sichuan Province,No.19PJ096the 1.3.5 Project for Disciplines of Excellence,West China Hospital,Sichuan University,No.2020HXFH008 and No.ZYJC18003.
文摘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.
文摘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</span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> and </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">4</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">month</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">s</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> female child, </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">diagnosed</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> 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.</span></span></span><span><span><span style="font-family:""> </span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">This case of PG is presented in the intention of increasing awareness of this uncommon condition with may oc</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">c</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ur</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;">ring</span></span></span><span style="font-family:Verdana;"><span style="font-family:Verdana;"><span style="font-family:Verdana;"> in association with haematological malignancy. PG may precede, develop concurrently with, or follow various conditions</span></span></span><span style="font-family:Verdana;">.
文摘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.
文摘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).
文摘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.
文摘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.
基金supported by research grants(to B.S.)from The Broad Foundation and The Crohn’s and Colitis Foundation of America.
文摘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.
