Vol 5 No 12

Remitting seronegative symmetrical synovitis with pitting oedema (RS3PE) is a rare syndrome that affects the elderly. Although the aetiology is not fully understood, it has been related to multiple diseases including cancer. We present the case of an 80-year-old man with a full spectrum of signs and symptoms compatible with RS3PE: sudden onset of bilateral polyarthralgia of the metacarpophalangeal and proximal interphalangeal joints, oedema of the dorsum of the hands, increased inflammatory markers and seronegative rheumatoid factor. After a 5-day course of corticotherapy, the patient became asymptomatic although maintaining a mild anaemia. During a search for the underlying cause of the RS3PE, an early stage adenocarcinoma of the caecum was diagnosed and surgically removed. No further treatment was performed and after 3 years of follow-up the patient remains without evidence of either paraneoplastic RS3PE or cancer.

Herpes simplex viruses are endemic worldwide, with an estimated seroprevalence of approximately 70% in developed countries. However, it is less well known that they are one of the viral causes of fulminant hepatitis (<2%) and constitute <1% of all causes of acute liver failure. We describe the case of an 89-year-old man who developed sepsis caused by a urinary tract infection due to drug-sensitive Escherichia coli. After empirical treatment with piperacillin-tazobactam was initiated, the patient’s condition worsened with shock, acute liver and renal failure, encephalopathy and persistent fever, that led to admission to the intensive care unit. The emergence of an acute abdomen prompted exploratory laparotomy but the patient died soon after surgery from abdominal haemorrhage. Immunohistochemical analysis of a liver biopsy specimen identified herpes simplex virus (HSV) hepatitis. The authors emphasize the need for better understanding of this rare condition in order to more precisely identify patients at risk who need more aggressive evaluation and empirical treatment, especially patients presenting with marked hepatic cytolysis with a rapidly worsening clinical evolution.

Acute non-rheumatic streptococcal myopericarditis (ANRSM) is a rare complication of an upper airway infection by streptococcus group A in developed countries. Cardiac involvement in bacterial infections must be adequately treated because it can lead to long-term complications. This case report describes recurrent ANRSM in an 18-year-old man, which illustrates how difficult and challenging the diagnosis of this disease can be.

Calciphylaxis is a rare condition characterized by the emergence of non-healing skin ulcers secondary to arterial calcification and thrombosis, typically diagnosed in patients with end-stage kidney disease (ESKD). When it develops in patients without ESKD, it is called non-uremic calciphylaxis (NUC). The latter is an even rarer diagnosis with an uncertain pathophysiology and a high mortality rate (52%), mainly due to sepsis (50%). Cutaneous biopsy is diagnostic. Therapeutic measures recommended for NUC are limited to wound debridement, analgesia, and control of infection and risk factors. Other therapeutic options exist but with a low level of evidence. We present the case of a 78-year-old woman with NUC in her lower limbs who died of sepsis. NUC is a therapeutic challenge lacking efficient strategies.

Mauriac syndrome, first described in 1930, is typically diagnosed in young patients with poorly controlled type 1 diabetes mellitus and growth retardation, delayed puberty, Cushingoid features, hypercholesterolaemia and hepatomegaly. However, the sole presenting feature of Mauriac syndrome can be hepatic glycogenosis in both adults and children. The mainstay of treatment for hepatic glycogenosis is strict control of glucose levels, with an excellent prognosis with improved glycaemic control. The authors present the case of a 22-year-old female patient with type 1 diabetes mellitus and a history of poor glycaemic control who was admitted with diabetic ketoacidosis (DKA). She complained of episodes of right upper quadrant abdominal pain associated with nausea and vomiting for the last 2 months with worsening in the last 48 hours. Physical examination was remarkable for short stature and tenderness over the hepatic area with a mildly enlarged liver. The patient had elevated liver enzymes and persistent hyperlactacidaemia despite DKA resolution. Liver imaging suggested diffuse fat infiltration. The clinical suspicion of hepatic glycogenosis was confirmed by liver biopsy. After glycaemic control was improved, liver enzymes normalized and the episodes of abdominal pain, nausea and vomiting subsided.

Enoxaparin is indicated for the treatment or prevention of many clinical disorders including deep vein thromboembolism, atrial fibrillation and mechanical valve thrombosis. It is one of the most commonly prescribed drugs in hospitals. However, haemorrhagic complications can occur, particularly in the elderly, patients with renal function impairment and patients with a very high or very low body weight. The authors describe the cases of three patients who had one or more risk factors for haemorrhagic complications, such as abdominal haematomas. The clinical presentation was similar in all three cases, with sudden-onset abdominal pain, an altered state of consciousness and hypotension. In all cases, investigation showed acute anaemia and large abdominal haematomas on imaging studies. A conservative approach was taken in the three patients, with suspension and reversal of anticoagulation, fluid resuscitation and red blood cell transfusion. Haemodynamic stability was achieved in two of the patients, but the third patient died.
The authors consider it is important to present these case reports because of the widespread use of enoxaparin, and the need for rigorous dose adjustment for renal function variations and body weight. We hope this article raises awareness of haemorrhagic complications in high-risk groups and propose protocols are introduced for dose adjustment and monitoring the efficacy of enoxaparin.

Spinal cord haematoma, or haematomyelia, is a rare condition caused by several unusual disease processes. Traumatic events, such as spinal cord injury and surgery or procedures involving the spinal cord, are the most important causes of spinal cord haematoma. Rarely, it is associated with anticoagulation therapy. Irrespective of cause, spinal cord haematoma is considered a neurosurgical emergency and must be treated promptly in order to prevent neurological sequelae. The authors describe the case of a 69-year-old patient taking warfarin in the therapeutic range for a mechanic mitral valve, who developed chest pain with cervical and dorsal radiation, and experienced sudden paraparesis of the limbs. A CT of the spine confirmed haematomyelia. A high index of suspicion, prompt recognition and immediate intervention are essential to prevent major morbidity and mortality from intraspinal haemorrhage.

A crazy-paving pattern is a non-specific radiological sign which is characterized by the presence of diffuse ground?glass attenuation associated with interlobular septal thickening and intralobular lines. It was initially described as a pathognomonic sign of pulmonary alveolar proteinosis. However, it can be also found in many other diffuse acute and chronic lung diseases including diffuse alveolar haemorrhage (DAH), a rare and life-threatening clinical syndrome which can be caused by many conditions, the most frequent of these being capillaritis associated with systemic autoimmune diseases.
In this case report, we describe an 82-year-old female patient with acute respiratory failure and bilateral pulmonary infiltrates with the characteristic crazy-paving pattern. The final diagnosis was isolated DAH induced by microscopic polyangiitis. The patient was treated with IV high dose prednisolone and cyclophosphamide and was mechanically ventilated. Nevertheless, her clinical status progressively deteriorated and she died after 3 days from acute respiratory distress syndrome.

Mucormycosis is a life-threatening fungal infection whose incidence has been rising recently, mainly due to the increasing use of immunosuppressive and corticosteroid treatment. In previous decades, mucormycosis was associated with a very poor prognosis as mortality was approximately 100%. Mortality rates reported in recent literature have only slightly improved despite the availability of targeted therapy with amphotericin B. Pulmonary mucormycosis is characteristically encountered in severely immunocompromised hosts, while rhino-orbital disease is often seen in individuals with diabetes mellitus. We report a rare case of fulminant pulmonary mucormycosis as an exceptionally rare complication of corticosteroid treatment in a 76-year-old patient with chronic obstructive pulmonary disease (COPD) and diabetes. The patient had presented with typical symptoms of an infective COPD exacerbation. The interesting aspects of our case were the absence of malignancy or immunosuppression, the isolation of Rhizomucor species, and the fungal invasion of the pleura and pericardium. Unfortunately, our patient died on the 49th day of hospitalisation, despite appropriate treatment.

Light-chain deposition disease is a rare paraproteinaemia characterized by deposition of monoclonal light-chain immunoglobulins with a non-amyloid structure, most frequently affecting the kidney. The authors present the case of a 58-year-old man admitted due to haemorrhagic shock caused by spontaneous splenic rupture. Investigation showed coagulopathy, homogeneous hepatomegaly with elevated cholestasis enzymes but normal bilirubinaemia, and a nephrotic syndrome with monoclonal kappa light chains. Liver and bone marrow biopsies revealed light-chain deposition disease. Splenic rupture as the presentation of a paraproteinaemia is rare. The diagnosis of light-chain deposition disease should be considered in case of a monoclonal plasma cell disorder with Congo-red negative deposits on histology.

Primary thyroid lymphoma is a rare cause of both thyroid malignancy and extra-nodal lymphoma. It typically presents as a rapidly enlarging goitre with compressive symptoms. Histological analysis of a biopsy specimen is necessary to confirm the diagnosis. Awareness of this disease is important for early diagnosis and appropriate treatment. A 55-year-old man was admitted due to a 3-month history of cervical enlargement, facial and periorbital oedema and dyspnoea, apparently due to a superior vena cava syndrome. However, cervical ultrasound showed asymmetrical thyroid enlargement, lobulated contours and a hypoechogenic texture. A neck CT scan showed calibre reduction of the superior vena cava. The diagnosis of primary thyroid lymphoma was confirmed by open biopsy of the thyroid gland. The patient started treatment with rituximab with clinical improvement.

Haemostatic abnormalities frequently occur during sepsis and are most often attributed to disseminated intravascular coagulation (DIC). We report the case of a patient with severe coagulopathy acquired during fulminant S. aureus sepsis. DIC was not present. This coagulopathy was most likely caused by S. aureus exotoxins forming inhibitory complexes with coagulation factor Xa.