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Extraosseous Ewing's sarcoma (ES), also referred to as extraskeletal ES, is a rare tumor of primitive cells that occurs in children, adolescents, and young adults. This tumor mainly involves the soft tissue of the extremities and thorax. We report a case of extraskeletal ES of the jejunum without associated skeletal location. A 26-year-old woman with no medical history presented to the emergency department with a 3-day history of a fever along with nausea, vomiting, and abdominal pain. An abdominal contrast computed tomography scan revealed a homogeneous bowel mass size 8.5 cm × 4.3 cm × 6.1 cm in the lower abdomen with invasion of the ileum is noted. Extraluminal free air and abscess formation size 10.6 cm × 5.2 cm × 8.3 cm is noted, suggestive of bowel perforation. After the surgery, the specimen was sent to the pathology department for examination. We received one segmental jejunum (25 cm in length and 6 cm in diameter of the lumen) that weighed 450 g in total. Sections revealed an ill-ulcerative mass measuring 6 cm × 4 cm × 0.5 cm with gray-to-brown coloration and extensive necrosis with perforation; the mass was located 15 and 7 cm in length from the bilateral cut ends. In addition, focal vascular proliferation, and infiltrate deeply to the serosa with mesenteric enlarged lymph nodes were observed. Furthermore, the mesenteric regional lymph nodes show metastatic sarcoma. The definitive diagnosis was based on the findings of histomorphology, immunohistochemistry, and molecular pathology.

Background/Objectives Paraneoplastic pemphigus (PNP) is a rare mucocutaneous disease with a high mortality rate. It is defined by polymorphic mucocutaneous manifestations, particular histological features, characteristic results of direct and indirect immunofluorescence examinations, presences of specific autoantibodies, and associations with underlying neoplasms. However, currently, there is no existing study regarding the characteristics of PNP patients in Taiwan. In this study, we report a case series and try to determine the specific presentations of PNP patients in Taiwan. Materials and methods PNP patients treated in a referral center in northern Taiwan from 1998 to 2012 were retrospectively recruited. The clinical manifestations, histopathological features, findings of direct and indirect immunofluorescence, results of immunoblotting, and all relevant clinical information were collected. Results Eleven patients were identified with an average age of 62 years. Polymorphic mucocutaneous manifestations were observed in almost all patients. The most common presentation was pemphigus-like lesions, followed by lichen planus-like lesions. All patients had recalcitrant oral mucosal lesions. Five and four patients had genital and eye involvements, respectively. The mostly associated neoplasm is Castleman’s disease, followed by malignant thymoma. Acantholysis is the mostly observed histological features, followed by lichenoid dermatitis and interface dermatitis. Depositions of immunoglobulins or complements on the surface of keratinocytes or along basement membrane zone were found in eight and seven patients, respectively. Respiratory symptoms presented in eight patients. Despite intensive treatments, seven patients expired. Conclusion PNP in Taiwanese patients has a high association with Castleman’s disease or malignant thymoma. Complete laboratory examinations and thorough investigations for occult neoplasms are mandatory to establish a diagnosis in patients with clinical suspicions of PNP.

Background: Linear immunoglobulin A bullous dermatosis (LABD) is a rare autoimmune subepidermal bullous disease. It is defined by continuous linear deposition of IgA in the basement membrane zone on direct immunofluorescence microscopy. The clinical presentations of LABD may mimic other diseases, and data in Taiwanese populations are still lacking. The current study aims to examine LABD status in Taiwan. Methods: We reviewed the database at our institute from 1995 to 2008. The gold standard for the diagnosis of LABD is based on continuous linear depositions of IgA in the basement membrane zone on direct immunofluorescence. Results: A total of 16 LABD patients were identified. Mean age at diagnosis was 55 years, and most (> 80%) occurred after the fourth decade. The trunk was most commonly involved (76%). However, in contrast to previous reports, the mucosal involvement was rare in our series (18%). Initial impressions were dermatitis herpetiformis in 8 patients (50%), bullous pemphigoid in 4 patients (25%), and vasculitis, varicella, and pemphigus vulgaris in the remaining 4 patients (25%). Four patients reported a history of drug ingestion shortly before the onset of the disease, and all recovered after discontinuing the offending drugs. One of them had griseofulvin-associated LABD, a case not reported previously. The other three drugs were rifampin, vancomycin and gemcitabine. Among the various regimens, dapsone (100 mg) twice a day achieved the best treatment response in the five treated patients. Conclusion: The rare and diverse presentations of LABD highlight the importance of our study results in aiding clinical diagnosis and planning treatment strategies.