A. Kettaneh

Localized scleroderma: a series of 52 patients.

BACKGROUND Localized scleroderma also called morphea is a skin disorder of undetermined cause. The widely recognized Mayo Clinic Classification identifies 5 main morphea types: plaque, generalized, bullous, linear and deep. Whether each of these distinct types has a particular clinical course or is associated with some patient-related features is still unclear. METHODS We report here a retrospective series of patients with localized scleroderma with an attempt to identify features related to the type of lesion involved. The medical records of all patients with a diagnosis of localized scleroderma were reviewed by skilled practitioners. Lesions were classified according to the Mayo Clinic Classification. The relationship between each lesion type and various clinical features was tested by non-parametrical methods. RESULTS The sample of 52 patients included 43 females and 9 males. Median age at onset was 30 y (range 1-76). Frequencies of patients according to morphea types were: plaque morphea 41 (78.8%) (including morphea en plaque 30 (57.7%) and atrophoderma of Pasini-Pierini 11 (21.1%)), linear scleroderma 14 (26.9%). Nine patients (17.3%) had both types of localized scleroderma. Median age at onset was lower in patients with linear scleroderma (8 y (range 3-44)) than in others (36 y (range 1-77)) (p=0.0003). Head involvement was more common in patients with linear scleroderma (37.5%) than in other subtypes (11.1%) (p=0.05). Atrophoderma of Pasini-Pierini was never located at the head. Systemic symptoms, antinuclear antibodies and the rheumatic factor were not associated with localized scleroderma types or subtypes. CONCLUSION These results suggest that morphea types, in adults are not associated with distinct patient features except for age at disease onset (lower) and the localization on the head (more frequent), in patients with lesions of the linear type.

Article originalCorticothérapie systémique et alimentation : suivi des recommandations diététiques et relation entre apports alimentaires et apparition d'une lipodystrophieSystemic corticosteroid therapy: patients’ adherence to dietary advice and relationship between food intake and corticosteroid-induced lipodystrophy

Purpose No data is available about: 1) the adherence of corticosteroid-treated patients to dietary advice provided by physicians; 2) the relationship between food intake and the corticosteroid-induced lipodystrophy (CIL).

Fièvre prolongée associée à une éruption cutanée diffuse révélant une méningococcémie chronique

INTRODUCTION Chronic meningococcemia is an unusual clinical presentation within the spectrum of infections due to Neisseria meningitidis. CASE REPORT We report a 32-year-old man who presented with a 15-day history of fever and maculopapular skin rash, in the absence of meningeal irritation or severe sepsis manifestation. Blood culture identified N. meningitidis. Clinical course was uneventful after antibiotic treatment was initiated. CONCLUSION Early diagnosis of chronic meningococcemia is crucial for optimal management of the patient and his/her contacts. Such a diagnosis should be suspected in the presence of the characteristic clinical triad (recurrent fever, skin rash and arthralgia), and this clinical presentation should be distinguished from systemic vasculitis as inadequate prescription of corticosteroids may be deleterious.

Effet à court terme des apports sodés sur la pression artérielle des patients recevant une corticothérapie systémique : étude prospective, randomisée, croisée

INTRODUCTION It is unknown if the level of dietary-sodium intake influences blood pressure in patients receiving systemic corticosteroids. METHODS Randomized, single centre, crossover trial involving patients starting systemic corticosteroid therapy and having initial blood pressure less or equals to 159/99 mm Hg. The first period of sodium regimen was randomized (<3 g/j versus >6 g/j) and each period of sodium regimen lasted 3 weeks. No washout period was performed. Blood pressure was recorded for each patient at inclusion and after 3 weeks and 6 weeks. Moreover, all patients were asked to record on a standardized questionnaire everything they ate during 1 week of each period regimen. Questionnaires were analysed by a dietician for mean daily energy and sodium intakes during each period. Mixed models were used to estimate the relationship between sodium intake and blood pressure variations. RESULTS Between June 2006 and June 2008, 49 patients were randomized, 24 in group 1 (first period regimen=salt<3g/day; women: 63%; mean age: 56+/-21 years; baseline prednisone dosage: 54+/-19 mg/day) and 25 in group 2 (first period regimen=salt>6g/day; women: 56%; mean age: 60+/-19 years; baseline prednisone dosage: 56+/-16 mg/day). Mean daily salt intakes were 2.5+/-1.8 and 9.3+/-1.9 g/day during the first period and 7.8+/-3.2 and 3.8+/-2.9 g/day during the second period, respectively for group 1 and group 2. Blood pressure variations were not significantly associated with daily salt intakes or with randomisation group. No order effect was evidenced. By comparison with baseline, systolic blood pressure increased by greater than 20 mm Hg at week 6 in five patients (2 in group 1 and 3 in group 2). CONCLUSION At short-term, sodium intake does not seem to influence blood pressure variations in patients starting systemic corticosteroids therapy.

Neutropénie auto-immune primaire avec autoanticorps antineutrophiles traitée avec succès par sirolimus

We report a 74-year-old man with severe chronic primary neutropenia (neutrophil count: 390 per millimeter cube) uncovered following surgery for perianal abscess collection. Clinical, laboratory and roentgenographic findings revealed no abnormality. Antineutrophil antibodies were positive in two consecutive serum samples. Under cyclosporine, neutrophil count reached 1970 per millimeter cube. However, this therapy was discontinued due to new onset of severe renal failure. After six weeks, neutrophil count was 950 per millimeter cube and sirolimus was started, resulting in renal function improvement and resolution of neutropenia.

Augmentation de CXCL10 dans le sérum au cours de la pneumopathie interstitielle de la sclérodermie systémique

INTRODUCTION CXCL10, a gamma-interferon-induced chemokine seems to play a relevant role in lung involvement that occurs in systemic sclerosis (SSc). The objective of this study was to assess the serum level of CXCL10 in interstitial lung disease (ILD) associated with SSc. METHODS Serum level of CXCL10 was assayed in 23 healthy volunteers (60.0 years; 58.0-67.3) and 29 SSc patients (63.1 years; 60.1-69.4) by ELISA method. Pulmonary function tests (PFTs), lung CT-scan and echocardiogram were also performed in the patients. Serum levels from patients and healthy controls were compared and a comparison among SSc patients between those with and without ILD, as documented by lung CT-scan, was also performed. RESULTS Median CXCL10 level from patients with SSc was significantly higher than that from healthy volunteers (110.0 pg/ml; 60.8-223.8 versus 52.0; 41.3-65.8; p<0.001). Fifteen out of the 29 patients had ILD on lung CT-scan; the median CXCL10 level from SSc patients with ILD was significantly higher than that from SSc patients without ILD (210.0 pg/ml; 115.0-307.5 versus 76.0; 55.0-110.0; p=0.02). CONCLUSION Our findings suggest that CXCL10 is specifically increased in the lung involvement of SSc and plays a role in scleroderma lung disease.

Infarctus hépatiques associés à un syndrome HELLP précoce et un probable syndrome catastrophique des antiphospholipides

INTRODUCTION Among microangiopathic disorders of pregnancy, catastrophic antiphospholipid syndrome (CAPS) is a maternal and fetal life-threatening disorder. Hepatic involvement of this multi-systemic disorder can be confused with HELLP syndrome, occurring usually later in the course of pregnancy. CASE REPORT We report a case of probable CAPS with hepatic disease in a pregnant woman at 13 weeks gestation, with antiphospholipid syndrome and biological features of HELLP syndrome. Unspecific hepatic imaging, well-described in our case allowed undelayed therapy. CONCLUSION CAPS and HELLP syndrome, both severe microangiopathic disorders, may be associated. Nosological distinction does not modify treatment strategy, which is a maternal and foetal emergency, but their overlapping requires aggressive and early management.

Communication brèveInfarctus hépatiques associés à un syndrome HELLP précoce et un probable syndrome catastrophique des antiphospholipidesLiver infarcts with early HELLP syndrome and probable catastrophic antiphospholipid syndrome

INTRODUCTION Among microangiopathic disorders of pregnancy, catastrophic antiphospholipid syndrome (CAPS) is a maternal and fetal life-threatening disorder. Hepatic involvement of this multi-systemic disorder can be confused with HELLP syndrome, occurring usually later in the course of pregnancy. CASE REPORT We report a case of probable CAPS with hepatic disease in a pregnant woman at 13 weeks gestation, with antiphospholipid syndrome and biological features of HELLP syndrome. Unspecific hepatic imaging, well-described in our case allowed undelayed therapy. CONCLUSION CAPS and HELLP syndrome, both severe microangiopathic disorders, may be associated. Nosological distinction does not modify treatment strategy, which is a maternal and foetal emergency, but their overlapping requires aggressive and early management.

Des anomalies unguéales

Une femme de 38 ans consultait en avril 2008 dans le cadre u suivi annuel d’un phénomène de Raynaud évoluant depuis la n de l’adolescence. En avril 2000, elle avait eu un rash malaire on infiltré, une acrocyanose des extrémités ainsi qu’un livedo ctif des poignets et des genoux. Au cours de l’été 2000, elle était articulièrement gênée par son acrosyndrome et était traitée par ifédipine 30 mg/jour remplacé par du losartan 50 mg/jour en aison d’une mauvaise tolérance. À l’automne 2001, elle avait eu n panaris ainsi qu’une recrudescence du phénomène de Raynaud onduisant à la reprise du traitement par nifédipine 30 mg/jour. u cours de l’hiver 2004, elle eut un gonflement permanent et ndolore des doigts avec fissures. On constatait alors la présence e télangiectasies du nez. L’hypocomplémentémie, connue depuis lusieurs années, persistait sur la fraction C3 (0,66 g/l, N ≥ 0,9), la raction C4 étant à 0,13 g/l (N ≥ 0,10). En avril 2008, alors que le hénomène de Raynaud persistait depuis l’hiver, l’examen trouvait es anomalies des ongles des troisième, quatrième et cinquième oigts de la main droite (Fig. 1) et du cinquième doigt de la main auche. Selon la patiente, il s’agissait du premier épisode de ce type t les lésions étaient apparues au cours de l’hiver précédent. La apillaroscopie révélait quelques dystrophies mineures en caducée t tortueuses ainsi que de rares hémorragies. Il n’y avait aucun lément en faveur d’une microangiopathie organisée spécifique ’une sclérodermie, d’une polymyosite ou d’une maladie de Sharp.

Salmonella bredney : une cause rare d’anévrisme mycotique

Mycotic aneurysms are rare, remain asymptomatic for a long time, and may be life threatening by their rupture if therapy is delayed. Historically associated with Streptococcus pyogenes and Staphylococcus aureus, they now frequently involve Salmonella species in elderly or immunodeficient patients, and complicate vascular investigation or surgical procedures. Frequently located in the abdominal aorta, they can also be found rarely in other location. Therapy associates antibiotics and surgical debridement with reestablishment of vascular continuity. We report a case of ruptured popliteal aneurysm with Salmonella bredney bacteraemia.