Weber-Christian disease(WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation,being the subcutaneo...Weber-Christian disease(WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation,being the subcutaneous adipose tissue the most frequent one,followed by liver,spleen,bone marrow and mesenteric adipose tissue.Systemic corticosteroids are first line treatment,but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation.We report herein a case with WCD showing orbital,mesenteric and ileocolonic involvement,which required surgical treatment and also developed postoperative recurrence.Symptoms were resolved by administration of thalidomide and,afterwards,infliximab.To our knowledge,this is the first report of Weber-Christian disease with luminal ileocolonic involvement,treated with infliximab.展开更多
目的:分析国人特发性收缩性肠系膜炎的临床特点,提高对该病的认识,避免误诊误治．方法:回顾性分析我院确诊的3例和近28 a 来国内期刊发表的33篇文献报道44例总共47 例特发性收缩性肠系膜炎的临床资料,总结其在发病年龄、性别、诱因、临...目的:分析国人特发性收缩性肠系膜炎的临床特点,提高对该病的认识,避免误诊误治．方法:回顾性分析我院确诊的3例和近28 a 来国内期刊发表的33篇文献报道44例总共47 例特发性收缩性肠系膜炎的临床资料,总结其在发病年龄、性别、诱因、临床表现、诊治、病变部位大小、预后等方面的特点．结果:特发性收缩性肠系膜炎好发于中老年, 男女比例相当．以腹痛和腹部肿物为主要表现,病变长径多>5 cm,以小肠系膜多见,其次是盲肠升结肠系膜,横结肠、降结肠、乙状结肠系膜和大网膜少见．结论:特发性收缩性肠系膜炎缺乏特异性的临床和影像学表现,极易被误诊．确诊主要依赖手术探查和病理检查．本病的预后好．临床上提高对该病的认识和病理检查是提高确诊率的关键．展开更多
文摘Weber-Christian disease(WCD) is an inflammatory disease whose main histological feature is lobular panniculitis of adipose tissue. The location of panniculitis determines the clinical presentation,being the subcutaneous adipose tissue the most frequent one,followed by liver,spleen,bone marrow and mesenteric adipose tissue.Systemic corticosteroids are first line treatment,but other options should be considered if systemic symptoms are observed or in case of refractory clinical situation.We report herein a case with WCD showing orbital,mesenteric and ileocolonic involvement,which required surgical treatment and also developed postoperative recurrence.Symptoms were resolved by administration of thalidomide and,afterwards,infliximab.To our knowledge,this is the first report of Weber-Christian disease with luminal ileocolonic involvement,treated with infliximab.
