A case of Turner syndrome complicated with Crohn's disease, erythema nodosum and Hashimoto disease]

A 26-year-old woman, who had had Turner syndrome from age 10 years old, had diarrhea, fever, joint pain, and erythema in the lower left leg. She was given a diagnosis of Crohn's disease, erythema nodosum, and Hashimoto disease. Systemic steroid therapy was very effective for both intestinal and skin lesions. It has been reported that half of inflammatory bowel disease patients with Turner syndrome have 46XiX (q) type chromosome abnormality, and this case also has this type of abnormality.     

Pediatric autoimmune hepatitis in a patient who presented with erythema nodosum: a case report

Background

Autoimmune hepatitis (AIH) is a form of chronic hepatitis with unclear causative factors and is characterized by immunological and auto-immunological manifestations. Several extrahepatic manifestations, such as other autoimmune disorders, are associated with AIH. AIH with dermatological conditions as the initial manifestation is rare. We report the case of AIH in which erythema nodosum (EN) was the first manifestation.

Case Presentation

An 8-year-old girl with several persistent dermatological lesions was referred to our hospital several months ago. Her skin had nodular, painful, dry, and erythematous lesions, predominantly on the extensor areas of both the legs, with some erythematous patches on her face. Physical examination revealed that she had hepatosplenomegaly as well. Skin biopsy indicated EN. The results of the laboratory tests showed increased levels of several liver enzymes. The patient''s International Autoimmune Hepatitis Group (IAIHG) score was a definite indicator of AIH. The results of liver biopsy indicated AIH. Other causes of EN and abnormal liver function were ruled out. The only obvious cause of skin lesions was chronic inflammation due to an autoimmune response. The patient was treated for AIH, and her skin lesions along with other signs and symptoms resolved.

Conclusions

AIH can present with protean clinical manifestations, and is thus associated with the risk of delayed diagnosis. Dermatological manifestations, including EN, could indicate a serious disease, and further investigation might be required. AIH should be considered as the possible diagnosis in such cases.     

Subretinal mass as a presenting sign of posterior scleritis: a case report

We report a case of an unusual clinical presentation of posterior scleritis. A 40-year-old woman suffered from sudden blurred vision in her left eye for 1 day. She had no systemic or autoimmune diseases. Fundus examination revealed a subretinal mass (10.5 x 6 mm in basal diameter) in the nasal-superior quadrant extending from the optic disc to the periphery. Overlying retinal striae were obvious. Optic disc hyperemia and mild edema were noted. Fluorescein angiography did not show dye leakage or tumor-feeding vessels. Ultrasonography showed high internal reflectivity of the subretinal mass and thickened sclera with T-sign. Under the impression of posterior scleritis, corticosteroids and nonsteroidal anti-inflammatory drugs were given orally and topically. After 4 weeks, the subretinal mass disappeared without any sequelae. The patient recovered full vision. This case suggests that posterior scleritis is among the differential diagnoses for a fundus mass. There has been no other similar report in Taiwan.     

A case of Q fever with erythema nodosum

Background

Acute Q fever is asymptomatic in 60% of the patients, while the reminder may present with fever, pneumoniae, and hepatitis. Skin manifestations are uncommon including transient punctiform rashes, purpuric, or maculopapular eruptions. Erythema nodosum have seldom been reported.

Case presentation

A 37-year-old female presented with fever for 1 month and skin lesions consists of erythematous painful nodule of the legs. Serological testing for Coxiella burnetii was positive. Treatment consisted with doxycycline for 2 weeks. Evolution was favorable. The patient completely recovered and had no evidence of skin lesion 1 month later.

Conclusion

Because of its nonspecific clinical presentation, Q fever with erythema nodosum is probably underestimated. Q fever should be evocated when facing unexplained erythema nodosum even if there is not other typical clinical manifestation of Q fever.

     

Rickettsioses presenting as major joint arthritis and erythema nodosum: description of four patients

Erythema nodosum and aseptic arthritis are recognized associations of rickettsial infections. However, they usually present with a febrile illness rather than with severe arthritis. We report three patients who presented with incapacitating major joint arthritis and one who presented with severe spondyloarthropathy in addition to major joint arthritis due to serologically confirmed Orientia tsutsugamushi and Rickettsia conorii infections. All of them had erythema nodosum and low-grade fever. They had rapid clinical response to doxycycline. The findings and conclusions in this report are those of the authors and do not necessarily represent the views of the United States Department of Health and Human Services or the Centers for Disease Control and Prevention.     

Small bowel adenocarcinoma presenting as a first manifestation of Crohn's disease: report of a case,and a literature review

Small bowel Crohn's disease has been related to an increased incidence of small bowel adenocarcinoma, but the total number of reported cases is small. We present an interesting case of a young male patient with nephrolithiasis in childhood, an atypical intermittent history of diarrhoea also since his childhood, who developed obstructive ileus and underwent an urgent operation. The operation revealed a stenosis of the ileum owing to a mass, which proved to be a small bowel adenocarcinoma. One month later, the patient underwent a curative surgical resection of the tumour with additional lymphadenectomy, followed by chemotherapy for 6 months. Since then, the patient had mild diarrhoea but enteroclysis was normal. Approximately 2 years after the resection of the tumour, a new ileocolonoscopy demonstrated deep ulcerations of the terminal ileum and the adjacent area of the colon as well as anal ulcerations. The new biopsy specimens were convincing for Crohn's disease. There were no signs of residual or relapsing cancer. There is growing evidence that Crohn's disease is one of the triggering factors for the development of small bowel adenocarcinoma. Underlying Crohn's disease should be suspected in a young patient with an atypical history of diarrhoea and small bowel adenocarcinoma.     

Unclassified connective tissue disease presenting as cardiac tamponade: a case report

This report describes a case of cardiac tamponade as the initial manifestation of unclassified connective tissue disease (UCTD). A 68-year-old Japanese woman was admitted to hospital because of dyspnea and edema. She had undergone a radical left mastectomy for the treatment of breast cancer 18 years before. On admission, bilateral leg edema, hepatomegaly, and a paradoxical pulse were noted on physical examination. The erythrocyte sedimentation rate was elevated and the C-reactive protein was 2.8 mg/dl. Antinuclear antibodies and anti-SS-A/Ro antibodies were present. The scl-70 and anticentromere antibodies were elevated. Chest radiography showed cardiomegaly. Echocardiography revealed a large pericardial effusion, but the pericardial fluid did not contain malignant cells or bacteria. She did not meet the diagnostic criteria for any known connective tissue diseases, so was diagnosed with cardiac tamponade due to UCTD. Prednisolone (30 mg/day) was administered, which resulted in a gradual resolution of the pericardial effusion. Although connective tissue diseases are known to cause pericardial effusion, cardiac tamponade as the initial manifestation of the disease in the absence of other symptoms is quite rare.     

Hypertrophic cardiomyopathy in a patient with Crohn's disease: a case report

Coronary artery dissection after blunt chest trauma has rarely been reported in traumatic cardiac injuries, but it may cause fatal or nonfatal myocardial infarction. We report a case of dissection of the left main coronary artery, which resulted in acute myocardial infarction 5 weeks after blunt chest trauma. Multidetector-row computed tomography performed prior to the onset of infarction depicted intimal flap and dissected cavities.     

Crohn's disease presenting as pyogenic liver abscess with review of previous case reports

A 40-yr-old male doctor from India presented with pyogenic liver abscesses as the first manifestation of Crohn's disease. The Crohn's disease itself was limited to the appendix and the adjacent cecum and could be diagnosed only 6 months after the presentation with liver abscess. This single case highlights three unusual features of Crohn's disease, and stresses the importance of meticulous search for a cause for pyogenic liver abscess when it occurs in an otherwise healthy adult.     

Crohn＇s disease and recurrent appendicitis： A case report

The clinical diagnosis of classic Crohn's disease (CD) of the small bowel is based on a typical history, tender right lower quadrant fullness or mass, and characteristic radiographic findings of the terminal ileum. Appendicitis may as well present with chronic or recurrent symptoms and this presentation may be confused with CD. We herein describe the case of a young teenage girl with a presumptive diagnosis of CD, who was ultimately diagnosed as having chronic nongranulomatous appendicitis. The literature on the subject is reviewed.     

Liver abscess as a rare complication of Crohn's disease: a case report.

Pyogenic liver abscess is a rarely seen extraintestinal complication of Crohn's disease. It has different features from other liver abscesses. Its clinical and laboratory findings are not specific and mimic the reactivation of Crohn's disease and diagnosis can be delayed. The radiological methods are very useful in diagnosis and treatment of liver abscess. In this paper, we present a patient with pyogenic liver abscess which developed in the course of Crohn's disease.     

空肠克罗恩病急性穿孔1例报告

病例 罗某,男,26岁,因突发腹部疼痛1天入院。患者1天前无明显诱因突感剑突下阵发性绞痛,随后变为满腹持续性疼痛,阵发性加重,伴恶心、呕吐,呕吐为胃内容物,非喷射性。患者既往反复腹痛,未行胃镜检查,在当地医院诊断为胃病,予以雷尼替丁等对症治疗,效果欠佳,平日时有便秘,大便干结,或有时解稀便。