AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pa...We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.展开更多
A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of ...A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.展开更多
Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but ...Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.展开更多
Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s diseas...Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s disease (CD). We report a case of AGG that led to diagnosis of underlying CD, namely Cutaneous Crohn’s disease (CCD). We guess that CCD appeared in the anogenital region is equal to AGG with CD.展开更多
SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic in...SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic involvement may occasionally be the initial symptom of WG. A case report of WG was described, which first presented as facial nerve palsy. The combination of facial nerve palsy, sensorineural hearing loss and otitis media was unusual. We should raise suspicion. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. The diagnosis of a WG is made clinically based on clinical findings, histologic confirmation and titres of cytoplasmic pattern antineutrophil cytoplasmic autoantibodies (c-ANCA)/ Anti-proteinase 3 (Anti-PR3). Immunosuppressive therapy with steroids, cyclophosphamide (CTX) is required for relief. A delay in diagnosis may lead to devastating sequelae, such as facial nerve palsy and hearing loss. WG is a challenging disease for otorhinolaryngologist.展开更多
文摘AIM: To review pediatric cases of orofacial granulomatosis (OFG), report disease characteristics, and explore the association between OFG and Crohn’s disease.
文摘We present the case of a young man with involvement of the gastrointestinal tract in the early phase of Wegener's granulomatosis. The patient presented at the emergency department with sudden onset of abdominal pain, nausea and vomiting. Radiography work up was negative for free air although ultrasound examination showed extraluminal intra-abdominal fluid. Exploratory laparotomy showed perforation of the jejunum. The bowel was vital except for this small segment of jejunum. A 5-cm long segment of jejunum was resected which revealed ulcerative inflammation accompanied by occluded arteries of the small intestine. Although intestinal perforation in Wegener's granulomatosis is uncommon, several cases have been previously reported. Intestinal involvement in the early phase of the disease is even more uncommon. This case combined with prev iously reported cases emphasizes the possibility of gastrointestinal manifestation early in Wegener's disease.
文摘A case is presented of Wegener＇s granulomatosis limited to the testis and epididymis, simultaneously, in a 69-year-old man. Orchiectomy was carried out through an inguinal incision under the presumptive diagnosis of a right testicular tumor. A hard, irregular mass occupied the upper testicle and a portion of the epididymal head was visualized. Histopathologic examination of the specimen showed granulomatous inflammation of the testis and epididymis with prominent angiocentric granulomata in the walls of arteries, veins and foci of fibrinoid necrosis, surrounded by palisading inflammatory cells with a few giant cells. The diagnosis of limited Wegener＇s granulomatosis was considered, although antineutrophil cytoplasmic antibody （c-ANCA） test was negative 2 weeks after orchiectomy. The patient showed an excellent response after local complete excision. He remains free of disease 18 months after orchiectomy.
文摘Well-studied therapies have proven to be effective in treating Granulomatosis with polyangiitis (formerly Wegener’s granulomatosis) (GPA). There has been considerable improvement in survival of patients with GPA but treatment related morbidity and mortality remains still high, particularly in patients with renal disease. We describe a case of 64-year old woman with recent onset GPA, who responds well to the initial cyclophosphamide based therapy but latter develops a fatal stroke. Infectious complications should be considered in patients with GPA who are on adequate immunosuppression but develop symptoms that may mimic a relapse. Aggressive diagnostic interventions should be undertaken to discriminate between an infection and a relapse of GPA.
文摘Anogenital granulomatosis (AGG) is a rare chronic idiopathic disorder presenting as a painless swelling of the vulva, labia, penis, and/or anogenital area. Some AGG cases have been diagnosed along with Crohn’s disease (CD). We report a case of AGG that led to diagnosis of underlying CD, namely Cutaneous Crohn’s disease (CCD). We guess that CCD appeared in the anogenital region is equal to AGG with CD.
文摘SUMMARY Wegener’s granulomatosisis (WG) is a multi-system disease characterized by granuloma formation and necrotizing vasciculitis. It typically involves the upper and lower respiratory tract and kidney. Otologic involvement may occasionally be the initial symptom of WG. A case report of WG was described, which first presented as facial nerve palsy. The combination of facial nerve palsy, sensorineural hearing loss and otitis media was unusual. We should raise suspicion. Early diagnosis is vital if unnecessary surgical exploration is to be avoided. The diagnosis of a WG is made clinically based on clinical findings, histologic confirmation and titres of cytoplasmic pattern antineutrophil cytoplasmic autoantibodies (c-ANCA)/ Anti-proteinase 3 (Anti-PR3). Immunosuppressive therapy with steroids, cyclophosphamide (CTX) is required for relief. A delay in diagnosis may lead to devastating sequelae, such as facial nerve palsy and hearing loss. WG is a challenging disease for otorhinolaryngologist.
