Wells' syndrome associated with ulcerative colitis: a case report and literature review

Wells' syndrome, also termed eosinophilic cellulitis, is a dermatologic condition of unknown etiology that occurs as recurrent patches or plaques mimicking infectious cellulitis. Histopathology reveals an eosinophilic infiltrate and characteristic flame figures. Previous reports have associated this syndrome with parasitic infections, arthropod bites, pharmacologic agents, surgery, and hematologic disorders. We present a case report of a patient with Wells' syndrome associated with newly diagnosed ulcerative colitis. The dermatosis erupted concurrently with flares of ulcerative colitis. Furthermore, treatment of the ulcerative colitis led to resolution of the skin lesions. To our knowledge this describes the first association between inflammatory bowel disease and Wells' syndrome and argues for a distinct relationship between the two.     

Propionibacterium acnes and SAPHO syndrome: a case report and literature review

OBJECTIVE: To describe the presence of Propionibacterium acnes (P. acnes) in a series of patients with SAPHO syndrome in which a bone biopsy has been carried out and to discuss the results comparing them to the data described in the literature. METHODS: In 6 out of 56 patients with SAPHO syndrome, a bone biopsy from osteitic lesion was carried out. This invasive investigation was performed only in those cases in which it was necessary to clarify the diagnosis. RESULTS: Of the 6 biopsies processed, P. acnes was isolated in only one case. No other infectious agents were identified. CONCLUSION: P. acnes is not often found in bone lesions of SAPHO syndrome. A bone biopsy may represent a procedure useful for corroborating the diagnosis or for excluding other diseases only in specific cases.     

Appendix adenocarcinoma associated with ulcerative colitis: a case report and literature review

Ulcerative colitis (UC) represents a risk factor for colorectal cancer, but the association between UC and appendix cancer is uncommon. A 60–year–old woman with a 5–year history of UC initially received medical treatment with mesalazine and prednisone with no satisfactory response; therefore surgery was indicated. The procedure was a total intersphincteric proctocolectomy with ileostomy. Histopathological analysis indicated adenocarcinoma from the cecal appendix, and chronic–active ulcerative colitis of the colon. In conclusion, surgeons and pathologists should examine every surgical specimen from patients with UC because of the possibility, although remote, of a neoplasic pathology. The appendix adenocarcinoma and ulcerative colitis may or may not be associated, same as colon cancer in patients with UC.     

SAPHO综合征一例报道并文献复习

目的 探索ＳＡＰＨＯ综合征的临床特点及诊断方法。方法 报道了一例ＳＡＰＨＯ综合征并对ＳＡＰＨＯ综合征的文献进行复习。结果 本例有手足掌面脓疱疮,双侧锁骨和第一前肋骨炎,骨肥厚。骨病理切片示慢性炎症改变,故确诊为ＳＡＰＨＯ综合征。结论 ＳＡＰＨＯ综合征临床可以见到,应注意和其他疾病鉴别。     

Primary biliary cirrhosis and ulcerative colitis： A case report and review of literature

AIM: To summarize the characteristics of patients suffered from primary biliary cirrhosis associated with ulcerative colitis. METHODS: To report a new case and review the literature. RESULTS: There were 18 cases (including our case) of primary biliary cirrhosis complicated with ulcerative colitis reported in the literature. Compared with classical primary biliary cirrhosis, the patients were more often males and younger similar. The bowel lesions were usually mild with proctitis predominated. While ulcerative colitis was diagnosed before primary biliary cirrhosis in 13 cases, the presentation of primary biliary cirrhosis was earlier than that of ulcerative colitis in our new case reported here. The prevalence of primary biliary cirrhosis among patients of ulcerative colitis was almost 30 times higher than in general population. CONCLUSION: Association of primary biliary cirrhosis with ulcerative colitis is rare. It should be considered in the differential diagnosis of hepatobiliary disease in patients with ulcerative colitis, and vice versa.     

Crohn's disease and the SAPHO syndrome during treatment with infliximab: a case report and review of literature

We report the case of a 40 year-old Turkish woman with severe ileal Crohn's disease associated with palmoplantar pustulosis and aseptic spondylitis, also known as the SAPHO syndrome. However, in the present case, the syndrome developed three months after starting infliximab (Remicade) therapy, which is generally used to treat the SAPHO syndrome. This case-report discusses the unclear relationship between Crohn's disease, the appearance of a SAPHO syndrome and treatment with infliximab.     

Basedow's disease and chronic ulcerative colitis: a case report and review of the Japanese literature

A case of Basedow's disease, that developed after successful treatment of ulcerative colitis with a total colectomy, is presented, along with a review of the Japanese literature on the coexistence of hyperthyroidism and ulcerative colitis. A 26-year-old man was referred to our department, complaining of general fatigue, appetite loss, and palpitation. At age 14, blood was discovered in his stool and a diagnosis of ulcerative colitis was made. Since then, he has been treated with salazosulfapyridine and prednisolone. On examination, mild exophthalmos and thyroid swelling were observed. Both serum free T3 and T4 levels were increased along with a positive TSH receptor antibody, while TSH was decreased. Scintigraphic and ultrasonographic examinations of the thyroid gland showed diffuse enlargement. Treatment with thiamazole relieved the symptoms and normalized the thyroid function. Although a high incidence of autoimmune thyroid diseases in association with ulcerative colitis has been suggested, only 6 cases of hyperthyroidism coexisting with ulcerative colitis have been reported in Japan. A common immunological process has been suggested to be implicated in the pathogenesis of this association, however, the exact mechanism remains unclear.     

Postinfantile giant cell hepatitis complicating ulcerative colitis: a case report and review of the literature

Giant cell hepatitis is common in the neonatal period. When present in adults, it is known as postinfantile giant cell hepatitis (PGCH). PGCH can arise in the context of viral, drug-related, and autoimmune disorders but, in many other cases, its etiology remains unclear. We report a case of PGCH occurring in the setting of autoimmune hepatitis and ulcerative colitis. This case highlights the close association between PGCH and autoimmune disorders and the need to recognize it as a hepatic complication of inflammatory bowel disease.     

Autoimmune hemolytic anemia in ulcerative colitis. A case report with review of the literature

Autoimmune hemolytic anemia is a rare complication of ulcerative colitis. This report chronicles a patient successfully treated with steroids, and reviews treatment in previously reported cases. The fact that 21% of patients recovered with steroid therapy encourages a conservative approach before surgery is considered.     

Sjogren's syndrome in a patient with ulcerative colitis and primary sclerosing cholangitis: Case report and review of the literature

Inflammatory bowel disease has been reported to co-exist with other autoimmune diseases. Sjogren's syndrome is an autoimmune disorder characterized by xerostomy and/or xerophthalmy. Sjogren's syndrome occurring in IBD has been very rarely reported.A 45-year old woman diagnosed ten years ago with ulcerative pancolitis and primary sclerosing cholangitis was referred to our outpatient IBD clinic because of xerostomy but not for xerophthalmy for the previous three months. The patient had been under azathioprine maintenance treatment (2 mg/kg) and achieved long-term disease remission for the past 4 years. Patient clinical examination and laboratory tests were unremarkable. Salivary gland biopsy and complete ophthalmologic investigation were performed and the patient was diagnosed with Sjogren's syndrome.Understanding sicca manifestations in IBD is difficult since the pathogenesis of this intestinal disorder is not yet clear. Of these complex autoimmune phenomena which occur along with IBD it is quite difficult to categorize concomitant Sjogren's syndrome as primary or secondary and literature is conflicting. The possibility of Sjogren's syndrome should always be considered and properly investigated in patients diagnosed with inflammatory bowel disease who develop a constellation of constitutional sicca symptoms.     

Diagnosing the SAPHO syndrome: a report of three cases and review of literature

SAPHO, an acronym for synovitis, acne, pustulosis, hyperostosis and osteitis, is a heterogeneous entity with myriad presentations and features overlapping with other entities. It is a differential in patients presenting with skin and bone symptoms, either singly or in combination. Often misdiagnosed radiologically as a malignancy or infection, the diagnosis is seldom thought of. We present three cases referred to us for evaluation of findings unrelated to the presenting symptoms. After evaluation, a ⁹⁹Tc bone scan was ordered, which showed the ‘bull’s head sign’ in all the three cases, confirming the diagnosis. We review the literature for SAPHO. It has a few features which point to its diagnosis and can help us to distinguish it from other seronegative arthritis. The clinician should be aware of this entity and should not hesitate to order a ⁹⁹Tc bone scan. We conclude that SAPHO is not rare, but rather, it is underdiagnosed. High index of suspicion is necessary for diagnosis. A ⁹⁹Tc bone scan is diagnostic and should be ordered in patients having any of the presenting features of the syndrome. We put forward the suggestion of using ⁹⁹Tc bone scintigraphy to define a ‘pre-MRI’ stage of ankylosing spondylitis.     

Colocolonic intussusception of a giant pseudopolyp in a patient with ulcerative colitis: a case report and review of the literature

Adult intussusception in the setting of inflammatory bowel disease (IBD) is a rare phenomenon. Giant pseudopolyps, while generally considered benign, may function as lead points for intussusception. Diagnosis and management of intussusception in the setting of IBD can be fraught with hazards. We report the case of a 27-year-old male, recently diagnosed with ulcerative colitis and giant pseudopolyps, who presented with colocolonic intussusception and obstruction. Diagnosis was confirmed using CT imaging and the patient underwent resection of the colocolonic intussusception without reduction. The following case underscores the challenges in managing adult intussusception in the setting of IBD and allows for a review of the literature to date. Resection of non-reduced intussusception, rather than endoscopic or enema reduction, should continue to be definitive treatment of patients presenting with this unusual problem.     

Adenocarcinoma below ileoanal anastomosis for ulcerative colitis: report of a case and review of the literature

BACKGROUND: Restorative proctocolectomy with hand-sewn ileoanal anastomosis and mucosectomy is warranted in patients with dysplasia and/or cancer on ulcerative colitis to prevent subsequent neoplastic changes in the retained mucosa. However, complete excision of the colonic mucosa cannot be obtained reliably. We report a case of anal canal adenocarcinoma after handsewn anastomosis with mucosectomy. METHODS: A 47-year-old patient, previously submitted to ileorectal anastomosis for colonic cancer on ulcerative colitis, underwent completion proctectomy and handsewn ileoanal anastomosis with mucosectomy for recurrent anastomotic cancer. Two years later, we submitted the patient to pouch removal with permanent ileostomy for a mucinous adenocarcinoma of the anal canal (T2N2Mx) found at follow-up pouch endoscopy. CONCLUSIONS: Only four cases of adenocarcinoma after handsewn anastomosis have been reported in the literature. This new case we report confirms that the risk of malignancy after ileoanal anastomosis with mucosectomy, although small, is real, despite the surgeon taking care with this particular step of the procedure. Careful surveillance is needed in patients with surgical treatment for long-term ulcerative colitis or dysplasia.     

A case of tracheobronchitis in ulcerative colitis: a review of literature

Introduction: Tracheobronchitis is a rare extraintestinal manifestation of ulcerative colitis (UC) and is often mis‐diagnosed and treated as asthma. Objective: We report a case of tracheobromchitis in a patient with long‐standing UC, who had been treated for asthma for many years with a poor response to standard asthma management. Only very few cases are reported and in the majority of them tracheobronchitis developed after colectomy. Methods: Subsequent investigations of unremitting respiratory symptoms in our patient revealed tracheobronchitis. His UC remained quiescent for more than 20 years on medical treatment having never required a bowel resection. Results: On thoroughly investigating the aetiology for his pulmonary pathology, it was thought to be an extraintestinal manifestation of UC, which makes this case noteworthy. Conclusion: Pulmonary complications can develop many years after the diagnosis of inflammatory bowel disease, without any sign of disease activity. In such cases awareness and a high index of suspicion are important to ensure early diagnosis and treatment, and further investigations to detect large airway disease is warranted. The pulmonary manifestations tend to respond well to steroid therapy, which may prevent or ameliorate permanent lung damage. Please cite this paper as: Kar S and Thomas SG. A case of tracheobronchitis in ulcerative colitis: a review of literature. The Clinical Respiratory Journal 2009; 3: 51–54.     

Aortitis syndrome associated with ulcerative colitis: report of a case.

A 43-year-old Japanese woman with asynchronous onset of ulcerative colitis and aortitis syndrome (Takayasu's arteritis) is reported. The possible relationship between the two disorders is discussed.     

Ulcerative colitis and Sweet's syndrome: a case report and review of the literature.

A 47-year-old man with a history of ulcerative colitis on prednisone and azathioprine was admitted to the hospital with a four-day history of fever, skin rash, arthralgias and leukocytosis. A skin biopsy demonstrated neutrophilic infiltration of the dermis that was consistent with Sweet's syndrome. He improved after several days with an increase in his prednisone and azathioprine. Sweet's syndrome is a rare cutaneous manifestation of inflammatory bowel disease, with approximately 40 cases reported in the literature. In a previously reported case of a patient with ulcerative colitis-associated Sweet's syndrome who was on azathioprine at the time of the skin eruption, the azathioprine was stopped, raising the possibility of drug-induced Sweet's syndrome. In the present case, the azathioprine was actually increased with complete resolution of the skin manifestations. This would support the theory that immunosuppressive therapy is the mainstay of therapy for this condition. In conclusion, Sweet's syndrome is a neutrophilic dermatosis that is rarely associated with ulcerative colitis. It may occur while on immunosuppressive therapy and responds to an intensification of immunosuppression.     

Intracranial manifestations in SAPHO syndrome: the first case report in literature

A 41-year-old woman with SAPHO syndrome presented with numbness of her left arm followed by a generalized seizure. Computed tomography and magnetic resonance imaging of the brain revealed a small ring enhancing lesion in the right parietal lobe with adjacent meningeal thickening and enhancement. Surgical removal and histopathology showed evidence of severe chronic sterile inflammation and no malignant cell. Symptoms recurred and a lesion were again detected on radiological follow-up, but improvement occurred following treatment with antibiotics and biphosphonate, achieving a low inflammatory activity, reduction in CNS lesions and relative clinical well-being. This is a first report in literature about the central nervous system involvement in a patient with SAPHO syndrome.     

Reye''s syndrome in the adult: case report and review of the literature

A 22-year-old male presented with a brief history of progressive encephalopathy. One week previously, he had developed an upper respiratory infection that resolved spontaneously and was followed by intractable vomiting. He had taken salicylates for several days during the viral syndrome. The diagnosis of Reye's syndrome was confirmed by hepatic histology. Aggressive conservative management was followed by complete metabolic and neurological recovery. There are fewer than 10 reported cases of Reye's syndrome in adults but this disease may be more common than is generally suspected. The diagnosis should be considered in patients presenting with emesis and obtundation, who have recently had a viral illness and exhibit elevated blood ammonia and transaminases with normal cerebrospinal fluid. Confirmation is achieved by liver biopsy. Therapy is directed toward aggressive reduction of increased intracranial pressure.     

TNF inhibitors to treat ulcerative colitis in a metastatic breast cancer patient:A case report and literature review

Adalimumab(ADA)is a tumor necrosis factor(TNF)inhibitor,used for the treatment of inflammatory bowel disease.Previous studies have reported an increased risk of cancer following exposure to TNF inhibitors,but little has been reported for patients with cancer receiving TNF-inhibitor treatment.We present a female patient with metastatic breast cancer and ulcerative colitis(UC)who was treated with ADA.A 54-year-old African American female with a past history of left-sided breast cancer(BC)diagnosed at age 30 was initially treated with left-breast lumpectomy,axillary dissection,followed by chemotherapy and radiation therapy.Years after initial diagnosis,she developed recurrent,bilateral BC and had bilateral mastectomy.Subsequent restaging computed tomography(CT)scan demonstrated distant metastases to the bone and lymph nodes.Three years into her treatment of metastatic breast cancer,she was diagnosed with UC by colonoscopy.Her UC was not controlled for 5 mo with 5-aminosalicylates.Subcutaneous ADA was started and resulted in dramatic improvement of UC.Four months after starting ADA,along with ongoing chemotherapy,restaging CT scan showed resolution of the previously seen metastatic lymph nodes.Bone scan and follow-up positron emission tomography/CT scans performed every 6 mo indicated the stability of healed metastatic bone lesions for the past 3 years on ADA.While TNF-αinhibitors could theoretically promote further metastases in patients with prior cancer,this is the first report of a patient with metastatic breast cancer in whom the cancer has remained stable for 3 years after ADA initiation for UC.