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Symmetric peripheral gangrene (SPG) is a symmetrical distal ischemic lesion on at least 2 or more extremities in the absence of proximal arterial obstruction and vasculitis. It is a rare and severe clinical entity. The aim of this study was to describe clinical symptoms, etiological agents and the management of SPG through a series of 4 cases.We included all cases of SPG hospitalized between 2000 and 2014. The inclusion criterion was the presence of distal ischemic damage at two or more sites in the absence of large vessel obstruction.Four patients (2 men and 2 women) were included. The mean age was 43.2±12 years. Two patients had a history of splenectomy. All patients had blackening of the tips of the fingers and toes. Three patients presented with septic shock. The etiology was bacteremia involving Streptococcus pneumoniae in two cases and a malignant form of Mediterranean spotted fever (MSF). In addition to specific antibiotics, we used a potent vasodilator (iloprost) in two cases and curative heparin therapy in two cases. The outcome was favorable in 3 cases, with regression of necrotic lesions. One case required the amputation of non-perfused necrotic fingers and toes.SPG can complicate MSF in some rare cases. Thorough and repeated skin examinations are essential to ensure timely diagnosis and treatment of GPS in order to improve the prognosis.

Haemophagocytic syndrome (HS) is a rare disease with a severe prognosis that is defined by clinical, laboratory and histopathological criteria. Infections represent the classical cause of HS. HS secondary to Mediterranean spotted fever (MSF) is rare with only a few cases being reported in the literature.We report two cases of HS secondary to MSF in 2 men aged 77 and 63 years presenting a febrile maculo-purpuric eruption with inoculation ulcer associated with laboratory abnormalities (cytopenia, elevated ferritin, hypertriglyceridaemia). Haemophagocytosis was present in 2 cases. Serology and PCR for Rickettsia conorii were positive and militated in favour of recent infection responsible for the diagnosis of MSF.The first case of HS was described in 1979. Sixteen cases of HS secondary to MSF are described in the literature. Cytopenia associated with hyperferritinaemia and hypertriglyceridaemia strongly suggests MSF complicated by HS. The prognosis depends on the time elapsed since diagnosis and host-specific factors. Immunosuppressants and antibiotics may be necessary to ensure healing.Rickettsioses can induce HS, and this potential complication with a severe prognosis must be known.

La fièvre boutonneuse méditerranéenne est une maladie infectieuse du groupe des rickettsioses due à une bactérie intracellulaire: Rickettsia Conorii. Les formes pauci symptomatiques et bénignes sont prédominantes. Les formes graves sont rares et de plus en plus rapportées dans la littérature récente avec une atteinte multi systémique sévère pouvant menacer le pronostic vital. Nous rapportons dans ce travail un cas très rare d’un patient de 52 ans admis en réanimation pour convulsions, état de choc septique et insuffisance rénale aiguë. Devant la découverte au deuxième jour de la prise en charge de lésions de type « chancre d’inoculation » et boutonneuses, le diagnostic de rickettsiose grave avec une atteinte multi viscérale sévère a été évoqué puis confirmé par une sérologie. Une antibiothérapie par doxycycline a été alors introduite. Le patient va développer cependant une nécrose tubulaire aiguë nécessitant des séances d’épuration extra-rénale. Il va développer rapidement une défaillance multi viscérale mortelle. La rickettsiose, par la vascularite microcirculatoire qu’elle engendre, peut donner des tableaux aussi sévères. Les mécanismes impliqués et les facteurs pronostiques éventuels sont discutés dans ce travail à travers une revue de la littérature.

Mediterranean spotted fever is an endemic rickettsiosis in southern France. We report here the case of a 2-month-old baby who developed rickettsiosis after a tick bite.A 2-month-old baby was hospitalized in the pediatric ward for fever with maculopapular rash extending to the palms and plantar surfaces and an eschar after a tick bite. Rickettsiosis serology examined after 48 h of fever was negative, but Rickettsia (spotted group) PCR taken from the lesion at the bite site was positive. A 1-week treatment with clarithromycin was started. Fever and rash disappeared 3 days after treatment initiation.Mediterranean spotted fever is endemic in southern France. It is, therefore, important to consider this diagnosis and search for tick bite signs or an eschar when a patient presents with fever and maculopapular rash. The treatment of choice consists in doxycycline or macrolides.Around the Mediterranean sea in particular, Mediterranean spotted fever should be considered as a possible cause of febrile disease with rash, to allow for a specific antibiotic treatment as fast as possible and to avoid dangerous complications, even though few cases have been reported below the age of 3 months.

We report the case of a 3-year-old patient with Mediterranean spotted fever (MSF). The patient was infected through a tick bite in the forests surrounding Paris. Treatment with azithromycin was started with good results. MSF is not exceptional and should be considered in patients with fever, rash, and eruptions even outside the usual infested areas.

Mediterranean spotted fever (MSF) due to Rickettsia conorii is the most important tick-borne disease occurring in North Africa. The first description of MSF was made by Conor and Brush in 1910 in Tunisia. Clinical diagnosis relies on the association of fever, rash and inoculation's scar during summertime. Prognosis in MSF is usually good, however malignant forms were described. These forms occur in patients with comorbidities. G6PD deficiency is a classic ground for severe forms of MSF. Myocarditis is an uncommon complication in MSF; only few cases were reported in the literature. We report a new case of myocarditis complicating MSF in a 15-year-old patient with G6PD deficiency. The patient presented with fever and rash, evocative of MSF; he reported chest pain and the electrocardiogram showed ST segment elevation in anterior leads. Troponin level was elevated. Echocardiogram showed left ventricular dysfunction with 40% ejection fraction. Serologic tests confirmed R. conorii recent infection. Antibiotic treatment with vibramycine and rifadine was started. Patient also received classic treatment of myocarditis with left ventricular dysfunction associating CEI, ß-bloquers and diuretics. Evolution was favourable with complete recovery of left ventricular function. Myocarditis is an uncommon but severe complication of MSF. Early diagnosis and treatment allow favorable evolution.

The Mediterranean spotted fever is a bacterial infection caused by Rickettsia Conorii mainly around Mediterranean basin. It is often considered like a trivial infection. However, severe forms with a high morbidity and high mortality risk have been described. These forms often associate with impaired consciousness, abnormal liver function, impaired homeostasis, pneumonia and acute renal failure. Several mechanisms of renal damage during the Mediterranean spotted fever have been reported, their pathogenesis remains speculative and the prognosis is determined by the type of renal disease and on early treatment. Thus, rickettsiosis should be considered in combination of acute renal, hepatic cytolysis and thrombocytopenia, allowing rapid achievement of a specific treatment.

Mediterranean spotted fever is an infectious disease due to Rickettsia conori, endemic in the Mediterranean basin. It is usually considered to be a benign disease; however, severe systemic manifestations have been reported in which neurological involvement occurs. These complications are rare in children.We report a case of a 14-month-old boy, who was hospitalized for meningoencephalitis after seven days of high fever and maculopapular rash. On clinical observation there were generalized maculopapular elements and an inoculation eschar tache noire at the right ear. The diagnosis of Mediterranean spotted fever was established by epidemiological and clinical features and was confirmed by elevated IgM antibody titres against R. conorii by indirect immunofluorescence assay. Despite treatment with macrolides and corticosteroids, severe neurological sequelae have remained including decreased vision and epileptic seizures.The physiopathology of Mediterranean spotted fever includes diffuse vasculitis with endothelial injury. Early diagnosis and specific antibiotic treatment may reduce the risk of complications.

In France, Mediterranean spotted fever due to Rickettsia conorii is mainly observed in the South-east. Rhipicephalus sanguineus, the brown dog tick, is the vector and the reservoir of the disease. Clinical diagnosis relies on the association of fever, rash and inoculation's scar during summertime. Pathological lesion is a diffuse vasculitis. Severity is often explained by a delay in diagnosis, but prognosis is good in pediatrics. Treatment relies on doxycycline and on macrolids in children under eight.

Rickettsiae are strictly intracellular bacteria belonging to the Rickettsiaceae family. They are transmitted by various arthropods species, which are either vectors or reservoirs for the bacteria. The specific association between Rickettsieae and the vector are extensively studied, moreover Rickettsieae associated diseases are part of a continuously evolving field. Nevertheless, some rickettsiosis, such as epidemic typhus, have been described since the 16th century. Emerging diseases related to these bacteria are being investigated as well as new species, the implication of which is not always clear in human pathology when they are discovered. Some of these diseases are benign, others may be potentially fatal. Clinicians must therefore be aware of them. These issues are reviewed.

Thromboembolic complications were uncommon in mediterranean spotted fever.We report a case of 55-years- old man who was admitted for mediterranean spotted fever. Two days later, the patient developed chest pain due to a pulmonary thrombosis confirmed by angio-CT. The outcome was good with heparin therapy. The investigation for another cause of thrombosis was negative.Mediterranean spotted fever is usually a moderately severe self-limited illness. Pulmonary thrombosis was uncommon associated with severe disease.

Boutonneuse fever is a bacterial infection caused by Rickettsia conorii. It occurs mainly in countries around the Mediterranean basin. Most cases are benign. However severe forms with major morbidity and a high mortality risk have been described. Severe forms often involve altered mental status, hepatic cytolysis, hemostatic disturbances, pneumopathy, and kidney failure. The causes of renal complications are unclear. The purpose of this report is to describe three cases of boutonneuse fever associated with acute kidney failure due to different underlying mechanisms, i.e., acute renal function failure, acute tubular necrosis, and extracapillary glomerulonephritis. While the first two mechanisms of kidney failure have been reported frequently in association with Boutonneuse fever, extracapillary glomerulonephritis has, to our knowledge, been mentioned only once. This case supports speculation that Rickettsia conorii has a toxic effect on glomeruli.

Rickettsia conorii is the etiologic agent of Mediterranean spotted fever that is endemic in Mediterranean.We report four cases of serious form of Mediterranean spotted fever with neurological feature. Two of them 56 and 62 years old had meningoencephalitis. Two others had cerebellitis, they are aged 43 and 56. The course has been favourable with ciprofloxacin.It is a benign well-known illness in spite of apparition of severe visceral complications, which can drag the death in 2.5% of cases. Neurological feature is more frequent and present in 28% of cases. Precocious treatment prevents this unfavourable evolution.

We report the case of a patient who presented an engigmatic chorioretinopathy. The rickettsial etiology was confirmed late. This case recalls the general ophthalmological features of Mediterranean spotted fever. The serology pattern and different clinical arguments led to diagnosis.

Boutonneuse spotted fever is a summer and late spring infection caused by Rickettsia Conorii mainly in Middle East countries adjacent to the mediterranean coast (1). Malignant forms have been described in debilitated patients associated with prerenal azotemia, acute tubular necrosis or renal vasculitis (2, 3). We report for the first time to our knowledge, the association of an extracapillary glomerulonephritis with a Boutonneuse spotted fever. This observation suggest that Rickettsia Conorii, as other bacteria, could participate to the development of glomerulonephritis.

115 sera from African individuals, including 66 HIV-1-infected individuals and 49 HIV-seronegative age-matched healthy controls, living in Bangui, Central African Republic, were screened for Rickettsia conorii antibody by indirect immunofluorescent assay; 53 (46%) sera had significant IgG to R. conorii titer, and 5 (4.3%) had significant IgM to R. conorii titer, without significant difference between HIV-positive and negative individuals. These findings indicate a great exposure to spotted fever group rickettsial infections in Bangui.

The history and clinical presentation of Mediterranean spotted fever in a 72-year-old male patient with polycythemia vera are described. The patient presented soon after arrival in Bordeaux (France) with fever, arthralgia and an erythematous maculopapular skin rash involving the palms and foot soles. The clinical course was complicated by pulmonary involvement resulting in dyspnea and hypoxemia. The patient denied tick bite but had contact with a dog. Antibiotic therapy with doxycycline resulted in rapid improvement of all clinical findings. A significant rise in antibody titer against Rickettsia conorii confirmed the diagnosis. The case is discussed in relation to other published cases in Switzerland and illustrates that this infection can be acquired outside an endemic area.

We report 25 cases of boutonneuse fever with, in the acute phase, an increase of plasma transaminases, triglycerides and apoprotein B levels, a decrease of total, HDL and LDL cholesterol and a decrease of apoprotein A. These changes disappeared 1 to 4 weeks after the beginning of treatment. They might be due to a reduced activity of lecithin-cholesterol acetyltransferase and lipoprotein lipase, probably caused by the hepatic and vascular disorders frequently found in this disease.

The authors report a case of mediterranean boutonneuse fever disclosed in a 6 year-old child presenting with severe neurological disorder, coma, hypotonia, sphincteral disorders, rash and eschar at the site of bite. Rise of antibodies detected by indirect immunofluorescence test (1/80----1/320) confirmed the diagnosis. Visualization of ischemic internal capsules at cerebral CT scan brought evidence of vascular lesions in rickettsial disease.

Actual knowledges of the biology of rickettsia, their pathogenicity and the diagnostic are briefly exposed. The description of sero-epidemic surveys in Bolivia, Botswana, Ethiopia, Greece and France shows that fifty years after the death of Charles Nicolle, rickettsial diseases are still a major health problem in numerous countries.

The purpose of this work was to determine the kinetics of antibodies in mediterranean spotted fever as determined by three serologic methods: indirect fluorescent antibody test, latex agglutination test and indirect hemagglutination test. No difference was noticed in the early kinetics but after 6 months, the latex agglutination test and the indirect hemagglutination test did not detect antibodies but the indirect fluorescent antibody test was still positive. This reaction is convenient for seroepidemiologic studies.

The Marseilles area continues to be an endemic region of mediterranean tick bite fever. During the period 1975-1978 40 cases were observed in two medical departments in Marseilles. Maximum occurrence was in August. The real incidence of the disease was difficult to appreciate as declaration is not always made eventhough obligatory. Clinical manifestations remained typical.

A severe case of boutonneuse fever was reported which was only diagnosed after death. The infectious syndrome and cutaneous eruption were associated with meningitis, encephalitis, hypoxaemia and thrombocytopaenia. The absence of an initial lesion ("tache noire") and the notion of recent German measles contamination explained the late diagnosis. Serious cases of boutonneuse fever were usually rare; they were better known as Rocky Mountain spotted fever, a rickettsial infection of the same group, the clinical symptoms of which were very similar and which gave the same proteus agglutination reactions as with boutonneuse fever. An earlier diagnosis, now possible thanks to immunofluorescent techniques using skin biopsies, should enable earlier treatment.

We report 5 confirmed cases of boutonneuse fever. All patients presented fever with a generalised maculopapular rash, and the tache noire at the site of the tick bite. Trimethoprim sulfamethoxazole failed to cure one patient. Treatment with oral oxytetracycline was effective in all cases.

The authors report the case of a 20 day-old neonate who presented with boutonneuse fever. According to their knowledge, this is the first reported case occurring in a neonate. In this child, the infection seemed to be unusually severe with rapid evolution and encephalic signs.

The indirect immunofluorescent technic, recommended by WHO, allows us to consider again, the problem of rickettsial diseases in Tunisia. This pathology, always actual, presents, also in epidemiological as in clinical aspect, a predominance for Mediterranean spotted fever by R. conori. Murin typhus by R. mooseri is still remained, but its incidence seems to be slight. The frequency of Q Fever must to be determined.

The Mediterranean spotted fever is always present in the south of France. The actual incidence is unknown. The disease appears in summer. The diagnostic is based on the association of fever, "black spot" and exanthema and/or a seroconversion. Indirect immunofluorescence is the most used technique. Some patients have severe complications: neurologic, cardiovascular, renal, thrombopenia. These cases look like Rocky Mountain Spotted Fever. The treatment is based on tetracycline.

Twenty nine patients with Rickettsiosis (Tick born fever, n: 22, Q fever, n: 7) has been treated with fluoroquinolones. These compounds was used alone n: 25 (Pefloxacin, n: 15; Ofloxacin, n: 10) or associated n: 4 (Pefloxacin + Rifampin). The general efficacy was excellent. Common Tick born fever and pulmonary forms of Q fever was fast cured. Severe Tick born fever (neurologic or polyvisceral forms, n: 5) and Q fever with long term hyperthermia, n: 4 cas always cured without complications but the time necessary to obtain apyrexia was often longer (6 cases).