[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.

Identification of a primarily neurological phenotypic expression of xeroderma pigmentosum complementation group A in a Tunisian family.

Xeroderma pigmentosum (XP) is a rare genodermatosis predisposing to skin cancers. The disease is classified into eight groups. Among them, XP group A (XP-A) is characterized by the presence of neurological abnormalities in addition to cutaneous symptoms. In the present study, we report a particular family with XP-A in which some members showed an atypical clinical presentation, i.e. unexplained neurological abnormalities with discrete skin manifestations. Molecular investigation allowed identification of a novel XPA mutation and complete phenotype-genotype correlation for this new phenotypic expression of XP-A.

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.

Leishmaniasis in central and southern Tunisia: current geographical distribution of zymodemes.

The authors report the identification of Leishmania strains isolated from the Centre and the South of Tunisia. 266 strains were isolated between 1998 and 2006 from human (n=221 strains) and dogs (n=45 strains) hosts. The isoenzymatic identification exhibits the presence of in total five zymodemes belonging to three Leishmanio complexes: Leishmania infantum, L. major and L. killicki. All strains isolated from human and canine visceral leishmaniasis belonged to L. infantum. zymodeme MON-1 was the only one isolated from canine visceral leishmaniasis. However, it is predominant in human visceral leishmaniasis beside zymodeme MON-24 which was detected in two provinces of the Centre (Monastir and Kairouan) and zymodeme MON-80 isolated for the first time in Kairouan province. Three complexes are responsible for human cutaneous leishmaniasis: L. major MON-25 is the parasite the most frequently found in its classic foci in the Centre and the South of the country. L. infantum MON-24 was isolated for the first time in a small locality of Sfax (southern Tunisia) showing the appearance of a new focus of L. infantum. L. killicki was isolated in its original focus of Tataouine and in two new foci of the central part of the country (Sidi Bouzid and Kairouan).

[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.

Usefulness of PCR in the diagnosis of cutaneous leishmaniasis in Tunisia.

We assessed the efficiency of a PCR method in establishing the diagnosis of cutaneous leishmaniasis (CL) in Tunisian patients. Four hundred and thirty specimens collected passively from patients with cutaneous ulcers suggestive of leishmaniasis attending health centres for diagnosis were included in the study. Dermal scrapings were analysed both by parasitological (examination of Giemsa-stained smears and in vitro cultivation) methods and by a genus-specific PCR detecting a fragment of the 18S rRNA gene. Microscopy revealed amastigotes in 245 samples (57.0%) and in vitro cultivation gave positive results in 88 cases (20.5%), whereas PCR detected Leishmania in 301 samples (70%). The sensitivities inferred from our results were 99.3%, 80.8% and 29% for PCR, microscopic examination and in vitro cultivation, respectively. The different forms of CL in this country are caused by three species of Leishmania and are treated with the same protocol. Of 303 well-documented cases in our study, 99% were probably caused by Leishmania major and 1% by Leishmania infantum. The lack of species-specific diagnosis is not known to affect treatment or prognosis in Tunisia. These data support the incorporation of PCR into diagnostic strategies for CL, particularly in Tunisia.