Eberspacher Chiara, Mascagni D, Fralleone L, Maturo A, Di Matteo F M, De Cristofaro F, Merletti D, Santoro A, Mascagni P, Pontone S, Pironi D
G Chir. 2019 Jan-Feb;40(1):39-43.
Inflammatory bowel diseases may be associated with many extraintestinal complications, that in some cases can represent the first onset of these disorders. In particular during the course of the disease, Ulcerative Colitis develops extraintestinal manifestations very frequently. One of the rarest is pyoderma gangrenosum, a noninfectious neuthrophilic dermatosis, that can involve most commonly legs but also other parts of the skin or mucosas. It can be idiopathic or associated with gammopathies, vasculitis, chronic arthritis or, like in our case, with inflammatory bowel disease and malignancies.
A 38-year-old man was referred to our Department with a colo-cutaneous fistula in the left quadrant of abdominal wall. In the anamnesis he reported a trauma during a soccer match three weeks before. Through a CT scan and endoscopy with biopsy an inflammatory bowel disease with a segmental colitis and stenosis was diagnosed. After medical therapy, an initial radiological drainage and a period of parenteral nutrition, he underwent a left hemicolectomy. Despite the previous endoscopic biopsy the histopathological examination put in evidence not only inflammatory disease (in particular Ulcerative Colitis) but also a colorectal tumor pT4pN0. After the full recovery before chemotherapy he has developed on the chest and on the abdomen some painful nodules, with central necrosis, one of those in contact with one of the ribs. Through TC and RM it was impossible to understand the precise nature of these skin lesions. With biopsy a pyoderma gangrenosum was diagnosed and treated until complete resolution.
Management of inflammatory bowel diseases can be a true challenge, not only for the intestinal manifestations, but also for all the other features not related to gut. In some cases the same patient can develop many complications, such as malignancies or rare cutaneous diseases. Despite the initial surprise for such a weird evolution in a same patient, from fistula to inflammatory disease to cancer and finally to pyoderma gangrenosum, to face every single complication following consolidated diagnostic and pathological paths has been the correct strategy for controlling the disease.
炎症性肠病可能与许多肠外并发症相关，在某些情况下，这些并发症可能是这些疾病的首发症状。特别是在疾病过程中，溃疡性结肠炎经常出现肠外表现。其中最罕见的一种是坏疽性脓皮病，一种非感染性嗜中性皮病，最常累及腿部，但也可累及皮肤或黏膜的其他部位。它可以是特发性的，也可与丙种球蛋白病、血管炎、慢性关节炎相关，或者像我们的病例一样，与炎症性肠病和恶性肿瘤相关。
一名38岁男性因腹壁左象限结肠皮肤瘘被转诊至我院。在病史询问中，他报告三周前在一场足球比赛中受过伤。通过CT扫描和内镜活检，诊断为患有节段性结肠炎和狭窄的炎症性肠病。经过药物治疗、初步的放射引流和一段时间的肠外营养后，他接受了左半结肠切除术。尽管之前进行了内镜活检，但组织病理学检查不仅发现了炎症性疾病（特别是溃疡性结肠炎），还发现了一个pT4pN0期的结直肠癌。在化疗前完全康复后，他的胸部和腹部出现了一些疼痛性结节，伴有中央坏死，其中一个结节与一根肋骨接触。通过CT和磁共振成像无法明确这些皮肤病变的确切性质。经活检诊断为坏疽性脓皮病，并进行治疗直至完全消退。
炎症性肠病的治疗可能是一项真正的挑战，不仅在于肠道表现，还在于所有与肠道无关的其他特征。在某些情况下，同一患者可能会出现多种并发症，如恶性肿瘤或罕见的皮肤病。尽管对同一患者从瘘管到炎症性疾病再到癌症，最后发展为坏疽性脓皮病这种奇怪的病情演变最初感到惊讶，但遵循既定的诊断和病理路径应对每一个并发症是控制该疾病的正确策略。
