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Infectious complications of sickle cell disease are common and can be serious and difficult to diagnose. Epidemiological aspects of these infections are well documented. The most common germ in children is pneumococcus followed by Haemophilus influenzae and minor salmonella. In adults gram-negative germs including minor salmonella are the most frequent. The purpose of this report is to describe a case of a Citrobacter freundii spondylitis with prevertebral abscess extending to dorsal and lumbar spinal areas. Diagnosis was made during work-up for persistent vaso-occlusive manifestations. Treatment consisted of percutaneous lumbar drainage associated with antibiotherapy.

Pyomyositis is a primary pyogenic muscular infection. It has been originally described in the tropics. Presenting signs and symptoms are not specific. It is frequently misdiagnosed in the early stages. We report the case of a 47-year-old woman with systemic lupus erythematosus treated with corticosteroids who presented a thigh pyomyositis evidenced by echography and MRI. Outcome was uneventful after antibiotics and surgical drainage. Pyomyositis is increasingly described among immunocompromised people, including lupus patients treated with corticosteroids., (2009 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.)

We report a case of 36-year-old woman, admitted for hypotonic tetraparesis. Laboratory tests revealed severe hypokalaemia, acidosis, hyperchloremia and alkaline urinary pH allowing the diagnosis of distal tubular acidosis. Additional investigations led to the diagnosis of primary Sjögren's syndrome associated with Hashimoto's thyroïditis. The evolution was favorable under potassium citrate alkalinisation, the corticosteroid therapy and hormonal substitution. Based on this observation, the pathogenesis of distal tubular acidosis during auto-immune diseases (Sjögren's syndrome, monoclonal hypergammaglobulinemia, hypothyroidism) was discussed as well as its consequences and management.

Introduction: The aetiological inquiry in the presence of systemic granulomatosis should consider some uncommon forms of tuberculosis infections. For instance, Poncet's tuberculous rheumatism is a rare entity subject to controversy., Case Report: A 32-year-old man presenting febrile polyarthritis associated with spontaneously vanishing erythematous papular skin rash. The tests revealed a biological inflammatory syndrome and mediastinal lymph nodes. The biopsies showed cutaneous and mediastinal adenoid granulomatosis. The cutaneous lesions resolved spontaneously. The culture of the ganglionic sample was positive. The evolution was favourable under treatment and the articular pain disappeared within a few days., Conclusion: Poncet's tuberculous rheumatism may underly evolving visceral tuberculosis. It's presence requires a search for tuberculosis when systemic granulomatosis with cutaneous and articular involvement may simulate sarcoidosis.

Unlabelled: We report a case of a serious myopathy related to hypothyroidism with pituitary enlargement and hyperprolactinemia., Case Report: Mr D.L 33 years old man suffering from myalgia, muscular weakness and cramps for six months. The laboratory check-up revealed a high serum creatin phosphokinase level, an autoimmune hypothyroidism, hyperprolactinemia and pituitary enlargement. The electromyogram was normal and the muscle biopsy showed no obvious inflammation. The outcome was favourable under L. thyroxin., Discussion: The frequency of myopathy during hypothyroidism ranges from 30 to 80%. The main symptoms related are weakness, muscular cramps and myalgia. However, hyperprolactinemia and pituitary enlargement described in this case are due to reactional mechanism. These several hypothyroid manifestations improve remarkably under L.thyroxin., Conclusion: Proximal myopathy may rarely be displayed as the sole manifestation of hypothyroidism. Therefore, it is recommended that hypothyroid myopathy should be taken into account during differential diagnosis of proximal muscle weakness.

Bilateral primary non-Hodgkin's lymphoma (NHL) of the adrenals is uncommon. Less than 70 cases have been described in the literature. The symptoms of disease are variable and depend on the tumor size and the presence of adrenal insufficiency . We report a case of large diffuse B-cell adrenal lymphoma discovered in a 40-year-old woman presenting bilateral lumbar pain. Hormonal exploration demonstrated adrenal insufficiency. Imaging explorations showed a large and bilateral adrenal mass. Percutaneous CT-guided biopsy of the adrenal and search for extension revealed primary bilateral adrenal lymphoma. After glucocorticoid substitution, treatment was based on a CHOP regimen chemotherapy; outcome was unfavourable after the second cure; the patient died from septic shock. The diagnosis of primary adrenal non-Hodgkin lymphoma should be investigated in patients with a rapidly growing bilateral adrenal mass associated with adrenal insufficiency.

Introduction: IgDkappa myeloma accounts for less than 2% of all myeloma cases. Orbital involvement is uncommon in myeloma and the authors suggest a possible predilection for IgD myeloma to involve the orbit., Case Report: A 67-year-old women was admitted for diffuse bone pain and painful ptosis of the right eye. The symptoms had progressed over approximately 1 year and were associated with a severe decline in general health. The biochemical, hematological, and radiological investigations suggested the diagnosis of an IgD kappa myeloma. Orbital x-ray showed destruction of the greater and lesser right wing of the sphenoid bone. Treatment was polychemotherapy. The course of the disease was very rapid and death occurred within 17 months from renal failure and amyloidosis., Discussion: Orbital involvement by myeloma occurs in 0.3% of cases and the majority are unilateral. The typical orbital symptoms include ptosis, diplopia, loss of vision, and pain. This was not considered to contribute to poor prognosis but may affect the functional prognosis.

Unlabelled: Hypoparathyroidism is very rarely diagnosed in patients with lupus., Case: A 39-year-old women had systemic lupus erythematosus known for 3 years. She was admitted for a paresthesia of the lower limb, depression, insomnia and 5 kg weight loss. The laboratory tests revealed hypoparathyroidism and no sign of disease activity. Treatment with oral calcium and vitamin D resulted in correction of the hypocalcemia and QT prolongation., Discussion: The combined diagnosis lupus-hypoparathyroidism has very rarely been reported. We suggest there may be a common underlying pathological process linking the two diseases. The occurrence of these diseases in the same individual may be related to an underlying genetic predisposition or secondary to the generalized autoimmune disease process.

Purpose: Gastrointestinal bleeding, an uncommon complication of polycythemia can be the inaugural sign in exceptional cases., Case Report: A 35-year-old patient was hospitalized for upper gastrointestinal bleeding. Physical examination and laboratory tests led to the diagnosis of polycythemia (Vaquez disease). Gastroscopy showed an hemorrhagic bulber ulcer. Outcome was favorable after chemotherapy and antinuclear treatment., Conclusion: Based on this observation and data in the literature we propose that this unusual presentation would suggest a relationship between polycythemia and ulcer disease.

Unlabelled: Ankylosing spondylitis is a very uncommon finding in patients with sarcoidosis. Thirteen cases have been reported in the literature. We report a new case. Observation. - A 40-year-old man had inflammatory low back pain since 1983 which formerly responded to nonsteroidal inflammatory drugs (NSAIDs). He developed dyspnea and skin rash in 1993. Physical exam found cervical and lumbar spine stiffness and violaceous and circular lesions on the forehead, nose and right cheek. Laboratory tests showed: ESR at 50 mm, increased serum angiotensin-1-converting enzyme, and negative HLA B27. X-rays showed sacroiliac ankylosis, cervical and lumbar syndesmophytes and cervical facet joint ankylosis. The plain chest x-ray showed an interstitial syndrome. Chest CT scan showed mediastinal adenopathies. Skin biopsy disclosed non-caseating epitheliod and giant-cell granuloma. Outcome was good with steroid therapy but back pain was only improved by NSAIDs., Discussion: - This association raises a diagnostical problem because spine involvement in sarcoidosis can mimic ankylosing spondylitis. It also suggests the hypothesis of a pathophysiological link between the two diseases.

Introduction: Hypocomplementemic urticarial vasculitis (HUV) described by McDuffie is a rare entity recently individualized among vasculitis. We report two new cases., Exegesis: Case 1: a 41-year-old woman presented in 1994 with inflammatory polyarthralgia, diffuse urticaria, fever, and weight loss. Biology showed proteinuria, positive rheumatoid factor with hypocomplementemia and negative immunological tests. Skin and renal biopsies showed leukocytoclastic vasculitis and extramembranous glomerulopathy, respectively. Outcome within steroid therapy was marked by alternating clinical improvement and relapses. Case 2: a 39-year-old woman presented in 1994 with inflammatory polyarthritis, diffuse urticaria, Raynaud phenomenon, cough and dyspnea. Chest x-rays and CT scan showed interstitial fibrosis and echocardiography revealed pericarditis. Biology showed positive rheumatoid factor with hypocomplementemia and negative antinuclear antibodies. Skin biopsy showed leukocytoclastic vasculitis. Corticosteroids and cyclophosphamide improved the patient's condition. McDuffie HUV is a disease with varied systemic manifestations. Its existence is still contested by some authors. Treatment is still empirical and depends on the clinical features. It is based primarily on corticosteroids., Conclusion: McDuffie HUV is a defensible entity among urticarial vasculitis because of its particular clinical and biological features.

Sweet's syndrome belongs to the group of neutrophilic dermatoses. We report the case of a 36-year-old man admitted for stiff neck and fever. He had a history of recurrent oral aphtous ulcers, orchitis, phlebitis, two episodes of febrile acute polyarthritis with interstitial pneumonia. He presented a stiff neck and a temperature of 40 degrees C for two days associated with an erythematonodular eruption of the right periocular region. Laboratory exams showed an inflammatory syndrome with hyperleukocytosis. Skin biopsy showed dermic neutrophilic infiltrates, confirming the diagnosis of Sweet's syndrome. The patient improved dramatically with corticosteroids: the temperature fell and neck stiffness and skin lesions disappeared. In light of this case with a rich cohort of extracutaneous manifestations, we reviewed the literature on the characteristics of Sweet's syndrome. This syndrome is commonly associated with inflammatory and neoplastic diseases.