A Case of Dermatomyositis in a Patient with a Neuroendocrine Tumor at the Ampulla of Vater / 대한내과학회지

Dermatomyositis is an autoimmune disease characterized by chronic muscle inflammation that results in specific dermatological signs and decreased muscle strength. It is known to have a strong association with malignancy. Most neuroendocrine tumors arise from the gastrointestinal tract, with less than 1% of cases occurring at the ampulla of Vater. While cases of dermatomyositis associated with neuroendocrine tumors have been reported internationally, to date there have been no cases reported in Korea. This case report presents a 33-year-old male who had undergone pylorus-preserving pancreaticoduodenectomy for a neuroendocrine tumor located at the ampulla of Vater, and had been followed at the hospital after his procedure. Three years post-surgery, the patient developed serious muscle weakness in his upper-right and lower-left extremities, and was subsequently diagnosed with dermatomyositis. This paper presents the first domestic case of dermatomyositis developing in the context of a neuroendocrine tumor at the ampulla of Vater. A literature review on this subject follows in the discussion.

Idiopathic Retroperitoneal Fibrosis Associated with Rheumatoid Arthritis in a Patient with Concomitant Chronic B Viral Hepatitis

Retroperitoneal fibrosis (RPF) is a rare, progressive disease characterized by chronic non specific inflammation of the retroperitoneum. Although the pathogenesis of idiopathic retroperitoneal fibrosis (IRF) remains unclear, IRF has been reported in association with autoimmune disorders. However, few cases of IRF associated with rheumatoid arthritis (RA) have been reported. We experienced a rare case of IRF in a patient with RA and chronic B viral hepatitis. A 39-year-old Korean man with RA and hepatitis B was referred to our hospital due to left hydronephrosis. An abdominal computed tomography (CT) scan and magnetic resonance imaging (MRI) showed a diffuse infiltrating retroperitoneal mass around the abdominal aorta and left ureter. The patient underwent intraureteral stent insertion and was treated with corticosteroid. Three months later, the follow up abdominal CT showed that the retroperitoneal mass had decreased in size. Herein, we report the first case of coexistent IRF, RA, and chronic B viral hepatitis with a literature review.

Knee Synovitis Mimicking a Septic Arthritis

Synovitis is the inflammation of the synovial membrane with unknown etiology which occurs in association with auto-immune inflammatory arthritis, mainly in rheumatoid arthritis. Synovitis manifesting as rapidly progressing monoarticular or pauciarticualr symptoms could make early diagnosis difficult, thus it could be misdiagnosed as other forms of arthritic diseases. We experienced a rare case of knee joint synovitis which initially manifested as mimicking a septic arthritis. A 58-year-old-male patient underwent renovascular embolization due to retroperitoneal hemorrhage which was developed after renal biopsy. Suddenly, the patient's left knee joint became swollen rapidly with redness and tenderness. Moreover, his right knee also became inflamed. Surgical irrigation and intravenous antibiotics had never worked on his knee joint inflammation, however administration of intermediate dose of steroid could decrease inflammatory signs dramatically. Synovitis in a large joint could be mistaken as a septic arthritis, delaying the right diagnosis. Thus, we report this case with literature review.

Typical 18-FDG-PET/CT Findings of Polymyalgia Rheumatica: A Case Report

Polymyalgia rheumatica (PMR) is an inflammatory rheumatic condition characterized by generalized pain and morning stiffness in the shoulders, hip girdle, and neck. Since the pathogenesis of PMR is still uncertain, the diagnosis of PMR depends on clinical features. There have been several studies regarding radiological tools for the diagnosis of PMR. Recent studies using 18-FDG-PET showed bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis in PMR patients. However, there was no report on the efficacy of 18-FDG-PET for diagnosis of PMR in Korea. Here, we are first reporting a case of a Korean patient with PMR, who had radiological findings including bursitis, synovitis, uptake in the spinous process and asymptomatic large-vessel vasculitis on 18-FDG-PET/CT.

A Rare Case of Pericardial Actinomycosis / 감염과화학요법

Actinomycosis is a common chronic suppurative and granulomatous infection caused by anaerobic or microphilic bacteria primarily from the genus Actinomyces. However, Actinomyces is a rare cause of pericarditis. We experienced a rare case of pericardial actinomycosis. A previously healthy 44-year-old man presented with 3 days of fever, chest pain, and clinical signs of congestive heart failure. Chest computed tomography showed pericardial effusion, pericardial thickening, and bilateral pleural effusion. A subxiphoidpericardiotomy was performed, and a histological specimen was taken from the pericardium. A histological section of the pericardium showed an actinomycotic granule (sulfur granule). His symptoms and signs improved after administration of piperacillin/tazobactam and steroids. He was uneventfully discharged on oral amoxicillin/clavulanate. He recovered fully with no recurrence after six months of follow-up.

An Adrenal Cortical Adenoma with Adrenal Medullary Hyperplasia / 대한내과학회지

The relationship between the adrenal cortex and medulla has been studied since the 1960s. Rarely, a patient with an adrenal cortical adenoma presents with the findings of pheochromocytoma. However, there has been no report of a case with the clinical features of pheochromocytoma showing the pathological features of an adrenal cortical adenoma with medullary hyperplasia on histological examination. We report a 59-year-old-man who was shown to have an adrenal cortical adenoma, with medullary hyperplasia, during a diagnostic work up for pheochromocytoma.

A Case of Dermatomyositis Presenting with Rhabdomyolysis

Rhabdomyolysis is caused by injury to skeletal muscle and it involves leakage of intracellular contents into the plasma. Rhabdomyolysis is an extremely rare manifestation of dermatomyositis. Dermatomyositis is a rare idiopathic inflammatory myopathy that is characterized by chronic inflammation of skeletal muscles and skin, resulting in muscle weakness. A 20 year old Korean male soldier presented with acute muscle pain, weakness and skin rashes over the face, neck and anterior chest. He received military training with carrying a radio set one week previouslyago. The patient was treated for rhabdomyolysis. However, the patient's symptoms did not improve. Muscle biopsy results suggested the diagnosis of rhabdomyolysis. Nevertheless, the features of skin and muscle inflammation raised the possibility of dermatomyositis. High dose steroid treatment was started, and then the symptoms and signs of muscle inflammation were improved. Rhabdomyolysis as the presenting sign of dermatomyositis has not been reported in Korea. Thus, we report on this case with a literature review.

A Case of Diffuse Muscular Calcification following Necrotizing Cutaneous Vasculitis in a Patient with Dermatomyositis / 대한류마티스학회지

Dermatomyositis is a systemic connective tissue disease affecting the skeletal muscles and skin. Necrotizing cutaneous vasculitis and subsequent diffuse subcutaneous and muscular calcification in patients with dermatomyositis is uncommon and has not been reported. A 23-year-old female with dermatomyositis developed necrotizing cutaneous vasculitis in the knee, elbow, buttock, toe and fingers associated with large ulcer formation. Her left 5th finger was amputated due to massive soft tissue destruction, and her right 5th finger later auto-amputated. Thereafter, she developed small subcutaneous nodules on both wrists, elbows, upper arms, neck, buttocks and thighs. X-ray, computerized tomography and gracilis muscle biopsy confirmed diffuse subcutaneous, fascia and muscular calcifications.