Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBD) in Tunisian children.We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for whom direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population.Thirty-one children with LAD were collected representing 65.9 percent of all AIBD of children collected in the same period, with a mean age of 5.5 years and a sex ratio M/F of 2.4. Most of the children had a generalized eruption (28/31) but more profuse on the face, pelvic region, buttocks, and limbs. Mucosal lesions were present in only 4 children (12.9%). The mean duration of the disease was 14 months. Direct immunofluorescence demonstrated predominantly linear IgA deposits along the dermal-epidermal junction in all patients. Faint IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67 percent of cases. Eight patients responded to Dapsone, but prednisone had to be added in 7 children and erythromycin in 4 others to control the disease. A long term remission period (34 months) was achieved in 61.9 percent of patients.This study confirms that LAD is the most common AIBD in children in Tunisia and it frequently occurs in preschool-aged males. Independently of the medication chosen for treatment, a long term remission is frequently observed.
Three clinical forms of cutaneous leishmaniasis can be found in Tunisia, i.e. the sporadic form due to Leishmania infantum in the North, the zoonotic epidemic form due to Leishmania major in the center and Southwest, and the chronic cutaneous form due to Leishmania tropica in the South. Unlike cutaneous forms, mucosal involvement is uncommon. The purpose of this report is to describe another case of mucosal leishmaniasis due to contiguity with a skin lesion in a 54-years-old woman. The patient responded well to treatment with meglumine antimoniate.
Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa.We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children.We present a 32-year retrospective study (January 1976 to December 2007). Children with chronic acquired bullous diseases seen at the Charles Nicolle Hospital of Tunis and for who direct immunofluorescence (DIF) of the perilesional skin demonstrated linear IgA immunoglobulin deposits were included in the study population.Thirty-one children with LAD were selected representing 65.9% of all AIBDs of children selected in the same period, with a mean age of 5.5 years and a sex ratio (M/F) of 2.4. Most of the children had generalized eruption (28/31), more profuse on the face, pelvic region, buttocks and limbs. Mucosal lesions happened in only four children (12.9%). The mean duration of the disease was 14 months. DIF demonstrated linear IgA deposits along the dermal-epidermal junction in all patients. IgG, IgM, and complement were also seen (20/31). Indirect immunofluorescence was negative in 67% of cases. Eight patients responded to dapsone; however, prednisone had to be added in seven children to control the disease and erythromycin in four others. A long-term remission period was achieved in 76.1% of patients.This study confirms that LAD is the most common AIBD in children in Tunisia which frequently occurs in preschool-aged males. Independently of the used drug, a long-term remission is frequently observed.
Agminated Spitz nevus arising on a background of nevus spilus (NS) is a rare condition. We report here a further case in a child that is original because it is induced by chemotherapy. A 3-year-old boy presented 3 months after the onset of a chemotherapy for a vesico-prostatic rhabdomyosarcoma, multiple pigmented papulo-nodules located on the face, neck, chest wall, and the higher back. These lesions have arose on a pre-existent large congenital histologically confirmed nevus spilus extending along the face, neck, the left shoulder and the left chest wall. Histological examination of three excised nodules led to the diagnosis of Spitz nevus. Our patient may have a high risk for melanoma since he has many criteria predisposing to this risk. Some of these criteria are related to NS but we should also take into account the chemotherapy induction and the high number of Spitz nevi.
Xeroderma pigmentosum Variant (XP-V) form is characterized by a late onset of skin symptoms. Our aim is the clinical and genetic investigations of XP-V Tunisian patients in order to develop a simple tool for early diagnosis. We investigated 16 suspected XP patients belonging to ten consanguineous families. Analysis of the POLH gene was performed by linkage analysis, long range PCR, and sequencing. Genetic analysis showed linkage to the POLH gene with a founder haplotype in all affected patients. Long range PCR of exon 9 to exon 11 showed a 3926 bp deletion compared to control individuals. Sequence analysis demonstrates that this deletion has occurred between two Alu-Sq2 repetitive sequences in the same orientation, respectively, in introns 9 and 10. We suggest that this mutation POLH NG_009252.1: g.36847_40771del3925 is caused by an equal crossover event that occurred between two homologous chromosomes at meiosis. These results allowed us to develop a simple test based on a simple PCR in order to screen suspected XP-V patients. In Tunisia, the prevalence of XP-V group seems to be underestimated and clinical diagnosis is usually later. Cascade screening of this founder mutation by PCR in regions with high frequency of XP provides a rapid and cost-effective tool for early diagnosis of XP-V in Tunisia and North Africa.
