Mohamed Ben Hmida

Manifestations cutanées chez les hémodialysés chroniques: Étude prospective de 363 cas

Resume Objectif Les manifestations cutanees chez les hemodialyses chroniques sont frequentes et variees. Leur pathogenie est souvent hypothetique. Le but de ce travail est d’evaluer la prevalence et la nature de ces lesions cutanees et de discuter certains mecanismes pathogeniques. Methodes Cette etude prospective a ete realisee du 1erfevrier au 30 avril 1996. Elle a interesse 363 patients hemodialyses dans 7 centres. Chaque patient a eu un interrogatoire et un examen dermatologique. Resultats Il y avait 210 hommes et 153 femmes. L’âge moyen etait de 50,6 ans. La duree totale de l’hemodialyse a varie de 1 a 192 mois avec une moyenne de 52,4 mois. Quatre-vingt huit pour cent des patients etudies avaient des manifestations cutanees et 44,9 % un prurit. Il est apparu apres le debut de l’hemodialyse chez 82,1 % d’entre eux. La xerose cutanee a ete trouvee chez 69 % des patients. Les alterations de la pigmentation cutanee ont ete observees dans 17 % des cas, representees essentiellement par une hyperpigmentation des zones photoexposees. Chez 6 patients, un blondissement de la peau, des cheveux et des phaneres est survenu 10 mois a 8 ans apres le debut de l’hemodialyse. L’hyperkeratose folliculaire a ete constatee chez 15 % des patients. Une seule patiente avait une pseudomaladie de Kyrle confirmee. Les troubles des phaneres representes essentiellement par les ongles amincis et les hemorragies sous-ungueales ont ete notes dans 18,5 % des cas. Les autres manifestations cutanees ont ete les petechies ou les ecchymoses (66 %), les infections cutanees a type de folliculite (9 %), les calcifications sous-cutanees (2 cas), une pseudoporphyrie cutanee (2 cas), un eczema en regard de la fistule (11,5 %) du essentiellement aux produits appliques localement. Conclusion Les manifestations cutanees chez les hemodialyses chroniques sont frequentes et polymorphes. Si la plupart sont connues de longue date, certaines, en particulier le blondissement, sont de connaissance plus recente. Leur pathogenie est le plus souvent mal elucidee. Le traitement est souvent symptomatique. La dialyse et la greffe renale constituent le meilleur traitement. Voir aussi dans ce numero l’editorial de Gerard Guillet, Les signes cutanes chez les hemodialyses: des marqueurs predictifs de morbidite ou de mortalite?.p 377-8.OBJECTIVE Chronic hemodialysis patients experience frequent and varied cutaneous manifestations, of often hypothetical pathogenesis. The aim of this work is to assess the prevalence and nature of these cutaneous lesions and discuss some pathogenic mechanisms. METHODS This prospective study was conducted from 1 February through 30 April 1996. It included 363 hemodialysis patients in seven centers. Each patient was interviewed and examined. RESULTS There were 210 men and 153 women. Their mean age was 50.6 years. The total duration of hemodialysis ranged from 1 to 192 months, with a mean of 52.4 months. 88% of the patients had cutaneous manifestations. Pruritus was reported by 44.8%. It appeared after hemodialysis began for 82.1% of them. Cutaneous xerosis was observed in 69%. Changes in pigmentation were observed in 17% of cases, primarily involving hyperpigmentation of photo-exposed areas. In 6 patients, skin, hair, and exoskeleton began to turn lighter 10 months to 8 years after hemodialysis. Follicular hyperkeratosis was observed in 15%. One patient had a confirmed case of perforating follicular dermatosis. Disorders of the exoskeleton (18.5% of cases) were represented mainly by thin nails and subungual hemorrhages. Other cutaneous manifestations included petechiae and ecchymoses (66%), folliculitis-type infections (9%), subcutaneous calcifications (2 cases), cutaneous pseudoporphyria (2 cases), and eczema around the fistula (11.5%), due essentially to locally-applied products. CONCLUSION Cutaneous manifestations in chronic hemodialysis patients are frequent and polymorphous. While most have long been known, lightening of skin, in particular, others have been discovered more recently. Their pathogenesis is most often poorly elucidated. Treatment is often symptomatic. Dialysis and renal transplantation constitute the best treatment.

Renal α‐smooth muscle actin: A new prognostic factor for lupus nephritis

Aim:  Systemic lupus erythematosus (SLE) is the prototype of autoimmune disease where renal involvement is frequent and always severe. Histological prognostic factors proposed for lupus nephritis (LN) including the World Health Organization and International Society of Nephrology/Renal Pathology Society – Working Group on the Classification classifications, active (AI) and chronicity (CI) indices may not predict response to treatment. The aim of this study was to correlate α‐smooth muscle actin (α‐SMA) expression, an early marker of glomerular and interstitial response to injury, to AI and CI, renal scarring progression and response to treatment.

Cutaneous abnormalities in patients with end stage renal failure on chronic hemodialysis. A study of 458 patients.

BACKGROUND Cutaneous manifestations occurring in patients with end stage renal failure on hemodialysis are polymorphic and diverse. OBJECTIVE The aim of our study was to assess the prevalence and characteristics of different cutaneous manifestations in patients on hemodialysis. PATIENTS AND METHODS We led a transverse investigation of all patients on hemodialysis in 12 haemodialysis centres of Sfax (Tunisia). We examined 458 patients (254 men and 204 women). The hemodialysis history ranged from 6 months to 24 years. A total of 394/458 (86%) patients had cutaneous abnormalities. These included pruritus (56.6% of patients), paleness (60.7%), xerosis (52.8%), hyperpigmentation or hypopigmentation (38.4%), venous dilation near the fistula (22.2%), eczema in the fistula area (14.8%), half-and-half nails (13.5%), onychodystrophy (6.1%), subungual hemorrhage (4.5%), leukonychia (4.5%), stomatitis (5.6%), xerostomia (3.2%), gingivitis (2.4%), uremic breath (2.1%), and skin calcificatins (0.4%). Nephrogenic fibrosing dermopathy was not detected in any of our patients. CONCLUSIONS Pruritus, paleness, dry skin as well as hyperpigmentation and hypopigmentation are the most frequent skin abnormalities observed in hemodialysis patients. The early recognition of some cutaneous conditions associated with end stage renal failure and hemodialysis may allow early therapeutic intervention and decrease morbidity.

Association purpura rhumatoïde de l'adulte et carcinome épidermoïde du poumon

INTRODUCTION Association between Schönlein-Henoch purpura and neoplasm can suggest the responsibility of tumour antigens in the genesis of the vasculitis. We report a new case of squamous cell carcinoma associated with Schönlein-Henoch purpura and we discuss the reality of this association. CASE REPORT We report the case of a 50-year-old man who presents Schönlein-Henoch purpura with a purpura of lower limbs, joint involvement, gastrointestinal lesions and IgA renal mesangial deposits. The patient received three intravenous methylprednisolone pulses followed with oral corticosteroids. Six months later, while the vasculitis was in remission, the patient presented a squamous cell lung carcinoma. He was treated by chemotherapy and local radiotherapy. At the late follow-up, the neoplasm was incompletely resolved. CONCLUSION The neoplasm could be responsible of the development of the Schönlein-Henoch purpura. The discovery of this systemic vasculitis in an elderly patient should warrant a deep screening for an occult neoplasm.

Wegener’s granulomatosis presenting as multiple bilateral renal masses: case report and literature review

Wegener’s granulomatosis (WG) is a disease of unknown etiology characterized by necrotizing granulomatous vascularitis. The upper and lower respiratory tract and kidney involvements are very common; however, its presentation as bilateral renal masses is unusual. We report a case of a 59-year-old female patient who presented with multiple bilateral renal masses. The patient presented with sinusal and ocular symptoms suggestive of WG, and positive antineutrophil cytoplasmic antibodies (c-ANCA) with an anti-PR3 pattern. Histopathologic examination of the renal biopsy specimen revealed granulomatous inflammation with vasculitis and fibrinoid necrosis. The patient management, including prednisone and cyclophosphamid, induced a marked improvement of the renal masses. This case illustrates that WG should be considered in the differential diagnosis of renal masses.

Alport syndrome and diffuse leiomyomatosis

Review on Alport syndrome and diffuse leiomyomatosis, with data on clinics, and the genes involved.

Living and cadaver donor transplant programs in the Maghreb.

In the Maghreb, organ failure constitutes a major public health problem, especially given the increasing number of patients with chronic renal failure and the high cost of care. In this study, we attempted to seek the recommendations, through a questionnaire, of various officials related to organ transplantation as well as leaders of ethics committees and religious groups in different countries of the Maghreb. The objective was to improve the rate of organ donation and transplantation. We received 36 replies (62%) within the prescribed time limit. In our survey, 83% of the respondents felt that living donor transplantation should be promoted initially, followed gradually by measures to increase cadaver donor transplantation to achieve a target of about 30 transplants with cadaver kidney donors per million inhabitants. To expand the donor pool, 83% of the respondents proposed to expand the family circle to include the spouse and in laws. To improve the cadaver donation activity, one should improve the organizational aspects to ensure at least 50 renal transplantations per year (100%) and provide material motivation to the treatment team proportional to the activity of organ donation and transplantation. Finally, 93% of the respondents suggested suitable moral motivation of the donors.

Minimal change disease in horseshoe kidney

The horseshoe kidney is a frequent urological birth defect. The most frequent complications are urinary tract infections, stones and hydronephrosis. The occurrence of glomerular disease in horseshoe kidney is rare. Therefore, we report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. A 22-year-old Caucasian man without personal or family medical history admitted to the pneumology department for a pulmonary artery embolism. In presence of a generalized oedema, a biological assessment was performed yielding intense nephrotic syndrome with urine protein excretion 22g/day. The abdominal ultrasound revealed a horseshoe kidney. Hence a scanno-guided kidney biopsy was taken yielding minimal change disease. High dose steroids were started, then gradually tapered with good response. Horseshoe kidney is the most common renal fusion anomaly, with a prevalence of 0.25% among the general population. The occurrence of glomerular nephropathy in horseshoe kidney has been reported in few cases. We report the first case of minimal change disease occurring in a patient with horseshoe kidney in literature. The mechanism of the association between the horseshoe kidney and these renal pathologies could not be explained in the previous reports. There is no literature data indicating a high rate of glomerulonephritis in horseshoe kidneys. The co-incidence of two renal diseases in this patient can be only a coincidence. The question that arises is whether this glomerulopathy is associated or not with this anatomical abnormality. Further studies are needed to answer this question.