[Acute localized exanthematous pustulosis on the face: 6 cases in Tunisia]. / Pustulose exanthématique aiguë localisée de la face : six cas tunisiens.

BACKGROUND: Acute localized exanthematous pustulosis (ALEP) is a rare and localized variant of acute generalized exanthematous pustulosis (AGEP). Only 15 cases of ALEP have been reported to date in the literature, with all cases following drug administration. We report 6 paediatric cases of ALEP occurring in springtime, with no associated drug administration in any case. PATIENTS AND METHODS: Over the last three years (2011, 2012 and 2013), we observed 6 cases of ALEP in 6 Tunisian children aged between 9 and 14 years. All cases were observed during the spring months. Diagnosis of ALEP was based in all cases on the EuroSCAR criteria and on the definition of ALEP proposed by Prange et al. A drug-related aetiology was ruled out in all cases, with exposure to a specific planned plant (Thapsia garganica) being retained as an aetiological factor in one case. DISCUSSION: Drug administration is the most frequent though not the sole cause of ALEP. The seasonal nature of this dermatosis may suggest other causes, mainly viral infection, plant contact or airborne allergens.

[Cholesterol ester fatty acid composition in Tunisian type 2 diabetics with and without cardiovascular complications]. / Composition en acides gras des esters de cholestérol chez des diabétiques de type 2 tunisiens avec et sans complications cardiovasculaires.

BACKGROUND: Lipid and glycemic imbalances are frequent disorders found in diabetes type 2. These disorders are influenced by dietary means. AIM: to investigate saturated fatty acids (SFA), polyunsaturated fatty acids (PUFS) and oleic acid of cholesterol ester fraction in non-insulin dependant diabetes mellitus without cardiovascular complications (NIDDM), non-insulin dependant diabetes mellitus with cardiovascular complications (NIDDMc) and healthy controls. METHODS: The composition of cholesterol ester fatty acids in 35 NIDDM, 33 NIDDMc and 32 controls were measured by gas-chromatography. Glycaemia and lipid profile were measured using commercial kits. RESULTS: Compared to NIDDM and to controls, NIDDMc showed a significant increase of different SFA (C12:0, C14:0, C16:0, C18:0). Oleic acid (C18:1) was significantly decreased in NIDDMc and NIDDM compared to controls (15,88 +/- 2,34 and 22,66 +/- 4,14 vs 28,18 +/- 2,90). Linoleic acid (C18:2) was significantly increased in NIDDMc compared to NIDDM and controls (52,59 +/- 5,50 vs 49,29 +/- 8,58 and 39,26 +/- 10,46). Linolenic acid (C18:3) and arachidonic acid (C20:4) were significantly decreased in NIDDMc compared to NIDDM and to controls. Linoleic acid (C18:2) / linolenic acid (C18:3) ratio was increased in NIDDMc. CONCLUSION: Linoleic (C18:2) acid excess intake found in our NIDDMc could emphasize arachidonic synthesis which is directly transformed while an inflammatory syndrome observed in coronary pathologies.

[Creation and report of the Tunisian Fanconi Anemia Registry (TFAR)]. / Création et rapport du registre tunisien de l'anémie de Fanconi (TFAR).

INTRODUCTION: Fanconi anemia (FA) is a genetically and phenotypically heterogeneous inherited disease. Many groups have established FA registries. In Tunisia, in collaboration with the Tunisian Fanconi Anemia Study Group (TFASG), we set up the Tunisian Fanconi Anemia Registry (TFAR). PATIENTS AND METHODS: We contacted all hematology and pediatrics departments to include their FA patients diagnosed between January 1983 and December 2008. The registry is available on the TFASG web site (www.fanconi-tunisie.net). RESULTS: Sorting the files brought out 142 patients belonging to 118 families. The mean age at diagnosis was 11 years. There was consanguinity in 86%, malformative syndrome in 91%, and pancytopenia at diagnosis in 69%. Of 28 patients, 95% belonged to the FANCA group. Androgen treatment was given in 109 cases and genoidentical bone marrow transplantation (BMT) in 27 patients. The diagnosis of a myelodysplastic syndrome was retained in 4%, acute leukemia in 6%, and a solid tumor in 2%. The median overall survival time in all patients is 17 years 5 months; it is significantly better in patients having received allografts (p=0.01). CONCLUSION: FA seems frequent in Tunisia, which is in part explained by the high consanguinity and endogamy in this country. Hematologic impairment is still the most frequent revealing circumstance of the disease. It is often severe or moderate and requires androgen treatment or bone marrow transplantation. BMT should be proposed to all patients with an HLA-compatible donor.