Linear IgA bullous dermatosis in tunisian children: 31 cases.

BACKGROUND: Linear IgA bullous dermatosis (LAD) of children is relatively frequent in Africa. AIM: We undertook this study to evaluate the frequency of this disease among autoimmune bullous diseases (AIBDs) in Tunisian children. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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 nevi arising on a nevus spilus after chemotherapy.

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.

[Systemic corticosteroids and their place in the management of hemorrhagic erysipelas]. / Place de la corticothérapie générale dans le traitement de l'érysipèle hémorragique.

BACKGROUND: Haemorrhagic erysipelas is a recently described clinical condition. AIM: Our aim is to study the clinical and evolutive particularities of haemorrhagic erysipelas treated with adjuvant corticotherapy. METHODS: It's a retrospective study of 6 cases of erysipelas of the lower limbs with serious local signs (purpura, bullae, petechia). RESULTS: Surgical examination as well as evaluation of muscular enzymes blood level were achieved in all cases. Adequate antibiotherapy and rapidly degressive systemic corticosteroids (0.5 mg/Kg daily) were prescribed. Fever, pain and cutaneous signs regressed rapidly. Haemorrhagic erysipelas is different from common erysipelas by the presence of serious local signs (bullae, purpura), by its bad response to adequate antibiotics and by its response to systemic steroids.

[Hereditary ichthyosis in Tunisia: epidemiological study of 60 cases]. / Ichtyoses herediatires en Tunisie: etude epidemiologique a propos de 60 cas.

BACKGROUND: Ichthyosis are a group of inherited keratinizing disorders. The cutaneous abnormalities may be isolated or associated with extra-cutaneous symptoms. AIM: To report the epidemiological and clinical profiles of patients with these genodermatoses from a hospital tunisian study. METHODS: A retrospective study of all cases of ichthyosis referred during a period of 5 years to the department of dermatology of Charles Nicole's hospital of Tunis. RESULTS: Sixty cases of hereditary ichthyosis were seen. The sex-ratio was of 0.5. Parental consanguinity was noted in 36 patients (60%). Seventeen patients (25.7%) had a positive familial history of ichthyosis. The clinical form of ichthyosis was determined in 52 cases. The nonbullous ichthyosiform erythroderma was observed in 25 patients (41.6%). Sixty patients presented an ichthyosis vulgaris (26.6%). The other forms of ichthyosis were rarely observed : 4 cases of X-linked recessive ichthyosis, 2 cases of lamellar ichthyosis and 2 cases of bullous ichthyosiform erythroderma. Two patients were born with collodion-like membranes. Two cases presented a complex syndrome. CONCLUSION: The NBIE, commonly considered as a rare form of ichthyosis, was the most frequently form seen in our study (41.6%), probably because of the high frequency of consanguineous marriages in Tunisia. The IV represents the most frequent form reported in the literature and was observed in 25% of our patients. The classification of some ichthyosis associated with other extracutaneous abnormalities (found in 2 of our patients) remains difficult.

[Fluconazole and treatment of onychomycosis. About 86 cases]. / Fluconazole et traitement des onychomycoses. A propos de 86 cas.

Systemic treatment of onychomycosis is based these last years on the new drug utilization aiming to decrease the length of the treatment and secondary effects particularly with fluconazole. The aim of our study is to determine the efficiency and the tolerance of the fluconazole (Funzol) managed to the dose of 150 mg per week in the treatment of onychomycosis. It is a multicentric and prospective study done to different department of dermatology in Tunisia. Are included adult patients with clinical and mycologic documented onychomycosis. They are treated during 12 to 24 weeks for the fingernail and 24 to 36 weeks for the toenail. The assessment of the efficiency and the tolerance of the drug was clinic and biologic. During this survey, 86 patients are included, 55 female and 31 male with mean age of 43.5 years. Onychomycosis seat to hands in 30 cases (34.8%) with a predominance of Candida species (73%), to toes in 68 cases (79%) witch due in 85% of cases to dermatophytic agent (85%). At 6 months, clinical cure rate is observed in 51% of cases. After 9 months, at the end of therapy, 84% of patients were judged clinical successes and culture was negative in 82% of cases. Some minimal secondary effects are signalled in 11% of cases to 6 weeks and 10% to 12 weeks not justifying the stop of the treatment. So this study confirms the efficiency and the good tolerance of fluconazole in the treatment of onychomycosis.

[Leg ulcer in a patient treated with hydroxyurea for polycythemia vera]. / Ulcère de jambe chez une malade traitée par hydroxyurée pour maladie de Vaquez.

INTRODUCTION: Causes of leg ulcers vary widely, although venous insufficiency and peripheral arteriopathy are most common. Ulcers are much rarer in patients treated by hydroxyurea. CASE: A 66-year-old woman who had been treated with hydroxyurea for polycythemia vera for four years came to our consultation a month after ulceration of the left lateral malleolus had begun. Symmetric peripheral pulses were present and there was no patent venous insufficiency. After two months of local symptomatic treatment, the size of the ulcer had increased. The possible involvement of hydroxyurea in the genesis and maintenance of this leg ulcer was then considered. Hydroxyurea was stopped, and the ulcer progressively grew smaller, finally healing completely two months later. DISCUSSION: We conclude that hydroxyurea was the primary cause of the malleolar ulcer, for it healed rapidly when this treatment stopped. Hydroxyurea causes cutaneous atrophy; when followed by microtrauma, associated with deterioration in DNA repair mechanisms, it may lead to the formation of persistent cutaneous ulcers. Hydroxyurea can also affect the microcirculation and induce tissular anoxia, which may also explain both the occurrence of cutaneous ulcers after microtrauma and their often painful character.