[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.

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.

[Vitiligo and quality of life: a case-control study]. / Vitiligo et qualité de vie: étude cas-témoins.

BACKGROUND: Vitiligo is a common and visible form of leukoderma that can adversely affect the quality of life of patients. The aim of this study was to assess the impact of vitiligo on the quality of life of patients. PATIENTS AND METHODS: This was a cross-sectional case-control study performed between 1 September 2000 and 31 December 2001. Sixty patients with vitiligo and 60 controls paired for age and gender were collated. Quality of life was assessed using the Dermatology Life Quality Index (DLQI). RESULTS: Mean patient age was 38.9 years. The sex-ratio M/F was 1.1. Vitiligo was generalized in 80% of cases. Quality of life was significantly impaired in patients and to a greater extent in women and in cases affecting more than 10% of the body surface. All aspects of quality of life were affected. CONCLUSION: Because of its visible nature, vitiligo can impair patients' quality of life and have marked psychological impact.

[Acute generalized exanthematous pustulosis due to fluconazole]. / Pustulose exanthématique aiguë généralisée induite par le fluconazole.

INTRODUCTION: Fluconazole (Triflucan(R)), a systemic triazole antifungal agent is largely prescribed and some cutaneous side effects have already been described. We report the first case of acute generalized exanthematous pustulosis due to this molecule in a patient with cutaneous candidosis. CASE REPORT: A 65 year-old-woman was treated with fluconazole (200 mg/day) for a persistent cutaneous candidosis infection on the buttocks. After the third dose, the patient presented with a pustular eruption with erythema located on her trunk and in her large skin folds. The eruption was associated with fever at 39 degrees C, asthenia and neutrophilia (9,000/mm(3)). The histologic examination and the negativity of microbiological cultures were consistent with the diagnosis of acute generalized exanthematous pustulosis. The eruption cleared with local steroids in about ten days. Nineteen days later, the same pustular eruption occurred but without fever nor neutrophilia. DISCUSSION: Clinical, biological and histological manifestations were consistent with the diagnosis of acute generalized exanthematous pustulosis due to fluconazole. According to the imputability criteria of Begaud et al., intrinsic imputability of fluconazole was possible (I2). According to the classification of the EuroSCAR study, it was certain. No similar case of recurrence had already been described after the withdrawal of the molecule. We believe this is the first case of acute generalized exanthematous pustulosis due to fluconazole (extrinsic imputability: B0).

[Eccrine angiomatous hamartoma: an atypical case]. / Hamartome angio-eccrine. Un cas de présentation atypique.

BACKGROUND: We report a case of eccrine angiomatous hamartoma. This rare and benign skin disease is histologically characterized by the proliferation of both eccrine glands and vascular structures. CASE REPORT: A 65 year-old man had developed during the previous 8 years post-traumatic, angiomatous, extensive and plurifocal lesions of the left lower limb. There was neither pain or local hyperhidrosis. The diagnosis of eccrine angiomatous hamartoma was made on histological evidence. DISCUSSION: This observation is original because of the onset of the disease after 50 years of age, its widespread and plurifocal character and probable triggering-off by minor local trauma. The classical surgical treatment of eccrine angiomatous hamartoma was not suitable and a laser-CO2 therapy was undertaken instead. The results were very discreet.