Improvement of Pyoderma gangrenosum and psoriasis associated with Crohn disease with anti-tumor necrosis factor alpha monoclonal antibody.

BACKGROUND: Infliximab is an anti-tumor necrosis factor alpha monoclonal antibody IgG effective in the treatment and maintenance of remission of active refractory Crohn disease and associated draining enterocutaneous fistulae. Multiple infusions of infliximab show promising results in patients with rheumatoid arthritis. Currently, there is limited clinical experience with infliximab, and no published reports exist on its use in cutaneous disorders. OBSERVATIONS: We describe 2 patients with Crohn disease and pyoderma gangrenosum and 1 patient with Crohn disease and psoriasis who were treated with infliximab for recalcitrant Crohn fistulae, with concurrent improvement in their skin diseases. CONCLUSIONS: These cases suggest that infliximab, a promising therapeutic agent for refractory Crohn disease and fistulae, may also be effective in the treatment of pyoderma gangrenosum and psoriasis associated with Crohn disease.

Gastric sarcoidosis.

Sarcoidosis of the gastrointestinal tract is uncommon even though involvement of the liver, spleen, and adenopathy are becoming recognizable entities on computed tomography (CT). Involvement of the stomach, the most common site of sarcoidosis of the gastrointestinal tract, is usually associated with pulmonary disease. The radiologic appearances of gastric involvement are variable. Positive biopsies may be obtained in a radiologically normal stomach. Ulceration resembling peptic ulcer disease may occur, and mucosal enlargement may be minor, diffusely nodular, or significant enough to mimic Menetrier disease. In its most dramatic form, a linitis plastica appearance resembling scirrhous carcinoma has been reported.

Esophageal dysmotility from a small esophageal granular cell tumor.

Although typically small and submucosal, esophageal granular cell tumors frequently produce dysphagia. Esophageal manometry and a cine-esophagram in a patient with a 7-mm-wide esophageal granular cell tumor and dysphagia showed esophageal aperistalsis and a hypertensive lower esophageal sphincter that relaxed normally with swallowing. This finding suggests that dysmotility may contribute to the dysphagia frequently attributed to this tumor. This tumor is commonly believed to originate from Schwann cells and has a propensity to encompass and disrupt nerves that could produce the abnormal motility. Esophageal manometry is recommended when a small nonobstructing granular cell tumor is found in the evaluation of dysphagia.

Cessation of recurrent bleeding from gastrointestinal angiodysplasias after aortic valve replacement.

Recurrent bleeding from gastrointestinal angiodysplasias associated with aortic stenosis ceased after aortic valve replacement in two patients. In one patient numerous gastrointestinal angiodysplasias disappeared, as shown by endoscopy, after aortic valve replacement. Valve replacement may be adequate treatment for gastrointestinal bleeding from angiodysplasia associated with aortic stenosis. Elective surgery for angiodysplasias should be postponed until after a trial period to ascertain whether bleeding stops after valve replacement.

Adenosquamous carcinoma of the liver arising in biliary cystadenocarcinoma: clinical, radiologic, and pathologic features with review of the literature.

Primary biliary cystadenoma, cystadenocarcinoma, squamous carcinoma, and adenosquamous carcinoma of the liver are rare tumors. We describe a middle-aged woman with recurrent fever and a clinical diagnosis of hepatic abscess who proved at laparotomy to have adenosquamous carcinoma of the liver arising in a biliary cystadenocarcinoma. The pathologic features of the tumor and findings on ultrasonography, angiography, and computed tomography are described in detail. The pathogenetic relationships of this unusual tumor to chronic biliary inflammation, biliary cystadenoma, and cystadenocarcinoma and to bile duct malformations (von Meyenburg complexes) are considered.

Colonoscopy as an adjunctive technique in evaluating acquired colonic narrowing.

Fiberoptic colonscopy is a useful adjunct to the barium enema in establishing the nature of colonic narrowing for which surgical intervention is being considered in patients who are not clinically obstructed. When the mucosa can be demonstrated to be free of disease, celiotomy and resection can be avoided. Even without significant narrowing, it is sometimes possible to limit the extent of resection by proving the process to be benign. On review of 500 consecutive colonscopic procedures, "colonic narrowing" was the indication or finding in 47 patients. Barium enema had been inconclusive in 34 of these, whereas colonoscopy was inconclusive in only seven. In 34 patients the decision concerning surgical amangement was influenced by colonoscopy, and the accuracy of the endoscopic findings in the majority was confirmed by biopsy, laparoscopy, or celiotomy. On the basis of radiographic and endoscopic studies, 29 patients were not explored and in none was there delay or failure in the diagnosis of malignancy. There were no complications or deaths. Three illustrative cases are presented with correlative endoscopic and radiographic material.

The endoscopy corner: reversible ischemic colitis--correlation of colonoscopic and pathologic changes.

Ischemic colitis is a well described complication following distal aortic replacement. The diagnosis is typically made by correlating clinical signs such as the occurrence of abdominal pain and bloody diarrhea with supporting radiographic changes. In this report, the diagnosis of ischemic colitis was confirmed on the basis of colonoscopic and associated histopathological findings. Colonoscopy is a useful technic in establishing the diagnosis of ischemic colitis, especially in patients whose clinical condition makes barium enema not feasible.

Antibodies to ribosomes in chronic active hepatitis.

A recently developed Farr-type radioimmunoassay for the detection of antibodies to tritium-labeled HeLa cell cytoplasmic ribosomes was evaluated as a serologic test in a variety of liver diseases. Ribosomal antibodies were detected in the sera of 31.4% of 70 patients with chronic active hepatitis (CAH) and primary biliary cirrhosis compared with only 4.2% of patients with chronic persistent hepatitis, acute viral hepatitis, alcoholic liver disease, and miscellaneous liver diseases (X2 = 17.89, P less than 0.001). The level of ribosomal binding activity was also significantly higher in sera of patients with CAH and primary biliary cirrhosis (15.4% vs 6.5%; t = 5.61, P less than 0.001). The antibodies were observed almost exclusively in HBsAg-negative CAH with autoimmune features and infrequently at lower titer in HBsAg-positive CAH or CAH without HBsAg or autoantibodies. Inhibition experiments with purified ribosomal RNA suggested that the ribosomal antibodies are in part reactive with ribosomal RNA. Ribosomal antibodies appear to represent another example of the exaggerated immune response associated with CAH and primary biliary cirrhosis.

Hyperviscosity syndrome attributable to hyperglobulinemia in chronic active hepatitis.

A young women with clinical and histological features of chronic active hepatitis was noted to have extremely high levels of immunoglobulin (14.6 g per dl). This was associated with the hyperviscosity syndrome, diffuse coagulation abnormalities, and renal insufficiency in the absence of severe liver disease. Correction of these features occurred with plasmapheresis before corticosteroid therapy was begun. A similar group of persons with very high gamma-globulin levels, described previously under the heading of "plasma cell hepatitis," may form a distinct and rare subgroup of chronic active hepatitis patients.

Serum lysozyme, serum proteins, and immunoglobulin determinations in nonspecific inflammatory bowel disease.

The serum levels of lysozyme, serum electrophoresis, and serum immunoglobulins were determined prospectively in 101 patients with ulcerative colitis, ulcerative proctitis, Crohn's disease, or nonclassifiable nonspecific inflammatory bowel disease. Although the mean serum lysozyme concentration of patients with Crohn's disease (10.5 +/- 6.8 microgram/ml) and ulcerative colitis (9.6 +/- 4.1 microgram/ml) performed by a standardized lysoplate method was significantly greater than normal controls (6.0 +/- 1.5 microgram/ml), the results did not correlate with the diagnosis nor with the degree of disease activity. Individually separated protein fractions and serum immunoglobulins also did not correlate with the serum lysozyme levels. This study indicates that measurement of the level of serum lysozyme in individual patients is not helpful in determining the cause or degree of activity of nonspecific inflammatory bowel disease.

Ulcerative esophagitis and colitis in a pediatric pantient with Behcet's syndrome. Response to steroid therapy.

The gastrointestinal manifestations of Behcet's syndrome including colitis and esophageal ulceration have been recognized in recent years. In the present case, a girl with Behcet's syndrome was followed since age 11 for oral, vaginal, perianal ulcers and neurological involvement. At age 16 she developed esophageal ulceration and colitis, was treated with steroids and improved. Endoscopic and radiographic findings are presented, previously reported cases are reviewed and treatment is discussed.