Immune-Mediated Necrotizing Myopathy: An Often Misdiagnosed Entity

Immune-mediated necrotizing myopathies (IMNM) are recognized as a subgroup of idiopathic inflammatory myopathies (IIM). IMNM are defined based on a combination of clinical presentation and laboratory studies, requiring a specific myopathological pattern on muscle biopsy for diagnosis. The authors describe a case of a patient with necrotizing myopathy, thought to be immune mediated, highlighting the challenge of its differential diagnosis. As clinical assessment and diagnostic tools sometimes fail to determine whether a necrotizing myopathy is immune mediated, leading to misdiagnosis and a compromise of the optimal therapeutic approach, distinguishing between IMNM and other myopathies is crucial.

Emergency emicolectomy for intestinal primary Aspergillosis in acute myeloid leukemia

ntestinal aspergillosis is an infection with a very high death rate especially in leukemic patients. Here we describe a case of a 46 years old woman with acute myeloid leukemia (LAM M5) who developed intestinal primary aspergillosis. This patient was diagnosed with LAM M5 through bone marrow aspiration and bone biopsy in March 2004. Symptoms of the disease were slight persistent fever, weight loss, asthenia, anemia, thrombocytopenia,and leukocytosis with high number of blasts in peripheral blood. After induction chemotherapy with ICE (Ifosfamide, Carboplatin, Etoposide), she developed neutropenia and high fever without apparent infective foci. She was treated with empiric antibiotic therapy, nevertheless she developed an intense diarrhea and ileo-cecal distention. Diagnostic exams didn’t show signs of a focal lesion. Despite the change in antibiotic treatment and the transfusions of granulocytes and blood cells, the patient developed extremely critical conditions with persistence of neutropenia and abdominal distention. A surgical treatment was decided at the time. We treated the patient with a two steps surgical procedure. The first step was a right abdominal ileostomy followed by improvement of general conditions and then the second step a right colectomy. The histological morphology confirmed necrotizing colitis with Aspergillus ife. At that time , treatment with voriconazole was started. The general conditions of the patient improved rapidly and we were able to treat the patient with other medical anti-leukemic therapies. The patient is now cured and in healthy state. We obtained a good clinical result as only in other few cases described in literature.

Evaluation of negative pressure vacuum-assisted system in acute and chronic wounds closure. Our experience

Negative-pressure therapy or vacuum-assisted closure (VAC) has been used in clinical applications since the 1940’s and has increased in popularity over the past decade. This dressing technique consists of an open cell foam dressing put into the wound cavity, a vacuum pump produces a negative pressure and an adhesive drape. A controlled sub atmospheric pressure from 75 to 150 mmHg is applied. The vacuum-assisted closure has been applied by many clinicians to chronic wounds in humans; however it cannot be used as a replacement for surgical debridement. The initial treatment for every contaminated wound should be the necrosectomy. The VAC therapy has a complementary function and the range of its indications includes pressure sores, stasis ulcers, chronic wounds such as diabetic foot ulcers, post traumatic and post operative wounds, infected wounds such as necrotizing fasciitis or sternal wounds, soft-tissue injuries, bone exposed injuries, abdominal open wounds and for securing a skin graft. We describe our experience with the VAC dressing used to manage acute and chronic wounds in a series of 135 patients, with excellent results together with satisfaction of the patients.

Pancreatic pseudocyst: case report and short literature review

We report a case of pancreatic pseudocyst secondary to acute necrotizing pancreatitis treated with open cystogastrostomy. Following a literature review, we stress the enormous benefits offered by modern diagnostic techniques, and especially imaging techniques, for the diagnosis and monitoring of this disease. Treatment should be delayed for at least six weeks, following which the drainage by open surgery offers the best results and lowest morbidity and mortality, followed by laparoscopy and endoscopy, indicated in particular cases and in patients where open surgery is contraindicated.

A Young Woman with Fever and Cervical Lymphadenopathy

Kikuchi-Fujimoto's disease is a self-limiting and rare disorder of unknown aetiology. The typical presentation includes fever, cervical lymphadenopathy and night sweats. Consequently, it is part of the differential diagnosis of infectious, lymphoproliferative and connective tissue diseases. Histology demonstrates necrotizing histiocytic lymphadenitis. Treatment is symptomatic with non-steroidal antiinflammatory agents, although there are reports of corticosteroid use in complicated cases. We present the case of a 23-year-old woman admitted to hospital for fever and cervical lymphadenopathies, and diagnosed with Kikuchi-Fujimoto's disease.

Anti-Signal Recognition Particle Myopathy in a Geriatric Patient

Anti-signal recognition particle (SRP) myopathy is a rare idiopathic inflammatory myositis that usually affects middle-age women, and is characterized by rapidly progressive proximal and symmetrical muscle weakness, elevated creatine kinase levels, severe necrotizing immune-mediated myopathy, presence of anti-SRP autoantibodies and poor response to steroid therapy. We report a geriatric case of a previously independent patient, presenting with slow onset of proximal paraparesis, myalgia and severe gait impairment. The patient was treated with steroid and azathioprine, with laboratory and pain response but modest muscle strength improvement. The clinical presentation of this unusual patient was atypical, which hampered the correct diagnosis.