[Allopurinol-induced DRESS complicated by hemophagocytic lymphohistiocytosis]. / DRESS à l'allopurinol compliqué d'un syndrome d'activation macrophagique.

BACKGROUND: Drug hypersensitivity syndrome is among the most severe drug hypersensitivity reactions and in rare cases it may progress to hemophagocytic lymphohistiocytosis. Herein, we report a case of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. CASE REPORT: An 18-year-old girl presented with maculopapular rash associated with cervical lymphadenopahy appearing 3 weeks after treatment with allopurinol. Her hemodynamic status at admission was unstable. Cutaneous examination revealed an itchy maculopapular rash, which was purpuric at certain sites, together with facial edema. The diagnosis of drug hypersensitivity was suggested and was confirmed by histological examination of a skin biopsy. Allopurinol was stopped. Two weeks later, however, eosinophilia was noted. Further, four days after discontinuation of allopurinol, in view of the laboratory signs of bicytopenia, hyponatremia, hypertriglyceridemia and hyperferritinaemia, as well as the presence of hemophagocytosis in bone marrow, a diagnosis was made of lymphohistiocytosis hemophagocytic syndrome complicating a drug reaction with eosinophilia and systemic symptoms. Moreover, viral serology tests were negative. The patient was given intravenous immunoglobulin and the outcome was good. DISCUSSION: The literature contains only very few reports of drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis. The incriminated drugs were vancomycin, lamotrigine and phenobarbital. To our knowledge, there has only been one report of allopurinol-induced drug reaction with eosinophilia and systemic symptoms complicated by hemophagocytic lymphohistiocytosis.

[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.

[Benzylthiouracil-induced antineutrophil cytoplasmic antibody-associated cutaneous vasculitis: a case report and literature review]. / Vascularite cutanée à anticorps anti-cytoplasme des polynucléaires neutrophiles induite par le benzylthio-uracile : à propos d'un cas et revue de la littérature.

INTRODUCTION: Vasculitis associated to antineutrophil cytoplasmic antibodies is a rare complication of therapy with antithyroid medication. They were mainly reported in patients treated with propylthiouracil and rarely with benzylthiouracil. CASE REPORT: We report a 22-year-old woman treated with benzylthiouracil for Graves' disease, who developed a vasculitic skin involvement. The presence of antineutrophil cytoplasmic antibodies with anti-myeloperoxidase specificity was documented. The discontinuation of benzylthiouracil was followed by a complete disappearance of skin lesions and of antineutrophil cytoplasmic antibodies. CONCLUSION: To our knowledge, only ten cases of antineutrophil cytoplasmic antibodies vasculitis induced by benzylthiouracil have been previously reported in the literature. Our patient was characterized by the occurrence of isolated cutaneous vasculitis, without renal involvement. Early discontinuation of benzylthiouracil may have prevented the occurrence of severe visceral complication.

Primary cutaneous aggressive epidermotropic CD4/CD8 double-negative cytotoxic T-cell lymphoma.

The classification of cutaneous T-cell lymphomas (CTCL) is based on clinical, histological and immunohistochemical findings. Diagnosis may be difficult given the significant heterogeneity of these tumours. We describe herein an original case of primary cutaneous aggressive epidermotropic CD4/CD8 double-negative cytotoxic T-cell lymphoma that illustrates the difficulty in classification.

Fixed drug eruption induced by atenolol.

Fixed drug eruption (FDE) is characterized by recurrent well-defined lesions in the same location each time the responsible drug is taken. We report here a case of multiple FDE induced by atenolol in a 48-year-old woman confirmed by positive patch test in previously affected sites. Beta-blockers-induced FDE are very rare. Only two cases had been reported in the literature. To the best of our knowledge, this is the first case reported of atenolol-induced FDE confirmed by a positive patch test.

Les effets indesirables medicamenteux : experience de l'unite de pharmacovigilance de Monastir

Introduction : Les effets indesirables medicamenteux representent une cause importante de morbidite voire de mortalite. Notre etude a pour objectifs d'analyser les aspects epidemiologiques; cliniques et chronologiques des effets indesirables et de cerner les differents medicaments impliques dans l'apparition d'effets indesirables. Materiel et methodes : Etude retrospective portant sur toutes les observations notifieesa l'unite de pharmacovigilance de Monastir pendant 45 mois (de avril 2004 a decembre 2007). L'imputabilite medicamenteuse a ete etablie selon la methode de Begaud et al. Resultats : Nous avons ete sollicites pour etablir l'imputabilite medicamenteuse de 277 evenements. L'origine medicamenteuse a ete retenue pour 157 patients (56;6). Il s'agissait de 77 hommes et 80 femmes. L'age des patients a varie de 1 a 87 ans avec une mediane de 39 ans. La majorite des effets imputes aux medicaments etaient des manifestations cutanees (70;7) suivies des effets indesirables hepatiques (10;2). Les autres evenements etaient a type de syndrome pseudo parkinsonien; des reactions anaphylactiques et hematologiques. Les beta-lactamines etaient les plus incriminees aux manifestations cutanees (35); suivies des autres anti-infectieux (30). Conclusion : Nous avons mis en evidence une predominance des effets indesirables cutanes et une implication accrue des anti-infectieux dans la survenue de ces effets

Self-healing Langerhans cell histiocytosis (Hashimoto-Pritzker disease): two Tunisian cases.

Self-healing Langerhans cell histiocytosis (SHLCH) is a rare self-limited variant of Langerhans cell histiocytosis that presents at birth or during the neonatal period. It was first described by Hashimoto and Pritzker in 1973. Subsequently, more than 70 cases have been reported in the literature. Regarding age of onset, SHLCH should be divided into congenital SHLCH and rare late-onset type. We report here two additional cases of SHLCH in Tunisian infants. We emphasize the need for long-term follow-up in such patients.