Ehlers-Danlos syndrome represents a group of inherited connective tissue disorders characterized by varying degrees of joint laxity, skin fragility and hyperextensibility, and a bleeding tendency. The essential defect is a quantitative deficiency of collagen. At present, 10 clinical forms have been recognized on the basis of extent and severity of the principal features, in combination with other abnormalities and the likely mode of inheritance. Type II Ehlers-Danlos syndrome is characterized by mild skin and joint manifestations, the latter limited to hands and feet, and inherited as an autosomal dominant pattern. We experienced a case of type II Ehlers-Danlos syndrome in 9-year-old girl with easy bruisability, skin hyperelasticity, thin, atrophic and a shiny scar, the so-called cigarette-paper scar on anterior side of lower extremities, and joint hypermobility limited to hand and feet. The diagnosis was made by characteristic clinical features and skin biopsy findings. A brief review of related literature was made.
Biopsy ; Child ; Cicatrix ; Collagen ; Connective Tissue ; Diagnosis ; Ehlers-Danlos Syndrome* ; Female ; Foot ; Hand ; Hemorrhage ; Humans ; Joint Instability ; Joints ; Lower Extremity ; Skin ; Wills

Peptic ulcer bleeding is a frequent cause of upper G-I bleeding. Endoscopic injection therapy with hypertonic saline-epinephrine (HS-E) is a cost-effective and widely used therapy for hemostasis; however, 1~3% of patients experience bleeding or perforation. A 70 year-old male patient was admitted with melena and hematemesis. An endoscopy on admission showed the presence of a 1 cm- sized well-demarcated ulcer with pulsatile bleeding at the lesser curvature of the distal antrum. Approximately 28 mL of HS-E solution was injected around the exposed vessel. Follow-up endoscopy performed 7 days later showed the presence of a 2 cm-sized newly developed mucosa necrosis at the lesser curvature of the pyloric channel, which was caused by ischemic necrosis of the gastric mucosa after HS-E injection. Endoscopic injection therapy with HS-E solution is a relatively safe technique, but mucosa necrosis can occur with the use of a dose of HS-E that is considered safe. We report a case of gastric mucosa necrosis following endoscopic HS-E injection therapy of a bleeding peptic ulcer.
Endoscopy ; Follow-Up Studies ; Gastric Mucosa ; Glycosaminoglycans ; Hematemesis ; Hemorrhage ; Humans ; Male ; Melena ; Mucous Membrane ; Necrosis ; Peptic Ulcer ; Stomach ; Ulcer

Cystic lymphangiomas are comparatively rare, benign tumors of lymphatic system and their histogenesis are uncertain. About 75% of theses lesions are in the neck, 20% are in the axillary region, and 5% are in the mediastinum, retroperitoneal region or groin. But retroperitoneal cystic lymphangiomas are very rare. Retroperitoneal cystic lymphangiomas are usually found incidentally during diagnostic procedures performed for unrelated clinical reasons or at surgery. Although retroperitoneal cystic lymphangioma is a benign lesion, it may cause significant morbidity due to its large size and its often invasive character with a strong tedency to secondary infection. They generally present as a palpable mass or abdominal pain and fever related to hemorrhage or inflammation of the cystic wall. The treatment of choice is surgical excision. We experienced a case of retroperitoneal cystic lymphangioma in a 4-year-old boy who had developed rapid abdominal distention and abdominal pain for 4 days. We have completely excised large cystic mass & histologically confirmed cystic lymphangioma. A brief review of literature was made.
Abdominal Pain ; Child, Preschool ; Coinfection ; Fever ; Groin ; Hemorrhage ; Humans ; Inflammation ; Lymphangioma, Cystic* ; Lymphatic System ; Male ; Mediastinum ; Neck

Behcet's disease is a chronic disease characterized by recurrent systemic vasculitis. The pathogenesis remains unclear. This disease involves various organs of the body and it can have a variety of symptoms with an unpredictable course. The diagnosis of Behcet's disease is made in patients with oral and genital ulcers and uveitis. In addition, gastrointestinal symptoms are frequently present; however, intestinal ulcers are infrequent. The most common extra-oral sites of gastrointestinal involvement are ileocecal and colon lesions. The lesions are typically resistant to medical treatment and frequently recur after surgical treatment. Here we present two cases treated with the monoclonal antibody anti-tumor necrosis factor (TNF) and regression of all symptoms.
Antibodies, Monoclonal ; Chronic Disease ; Colon ; Humans ; Necrosis ; Systemic Vasculitis ; Ulcer ; Uveitis ; Infliximab

Pyoderma gangrenosum (PG) is a vasculitic process, often associated with systemic diseases, including Takayasu's arteritis (TA), rheumatoid arthritis, and inflammatory bowel disease. Most cases of PG associated with TA have been observed in Japan. However, this association is not commonly observed in North American and European patients. PG is often refractory to therapy, requiring high dosages of glucocorticoids. Here, we describe a case of recalcitrant PG that was associated with TA and successfully treated with methotrexate.
Arthritis, Rheumatoid ; Glucocorticoids ; Humans ; Inflammatory Bowel Diseases ; Japan ; Methotrexate* ; Pyoderma Gangrenosum* ; Pyoderma* ; Takayasu Arteritis*