Search PubMed⌕ Search

Biomedical subjects

B Fazaa

Publications and source records attributed to B Fazaa.

At least 19 recordsLinked to original sources

[Mucosal localization of leishmaniasis in Tunisia: 5 cases].

INTRODUCTION: Three epidemic-clinical forms of leishmaniasis are found in Tunisia: the sporadic cutaneous form due to L. Infantumin in the North, the zoonotic cutaneous form due to L. Major in the Center and South-West, and the chronic cutaneous form due to L. Tropica in the South. We report 5 cases of mucosal leishmaniasis diagnosed in a Dermatology unit in Tunis. OBSERVATIONS: Four women and one man, from the North-west of Tunisia, with a mean age of 42.4 years (range: 8-75 years) presented with leishmaniasis. The lesions were localized on the mucosa of the lips in 4 patients and on the endonasal mucosa with infiltration and nasal obstruction in a 75 year-old female patient. Diagnosis of leishmaniasis was established on direct examination in 4 cases and histological examination in 3 cases and by culture in NNN milieu for one patient exhibiting a MON5 L. Major leishmaniasis. All the patients responded well to treatment with intramuscular meglumine antimoniate (Glucantime). In our 5 patients, the mucosal involvement was not as mutilating, nor resistant to treatment, as that described for the cutaneous-mucosal forms in the New World. COMMENTS: Mucocutaneous leishmaniasis, endemic in Central and South America, are due to L. Braziliensis. They provoke mutilating and disfiguring lesions, resistant to treatment. In Tunisia, the forms of the disease observed are dermotropic, usually responsible for cutaneous leishmaniasis. However, mucosal involvement is not uncommon and is characterized by the absence of mutilating lesions and the excellent response to treatment.

Adolescent↗

[A whole family affected by xeroderma pigmentosum: clinical and genetic particularities].

INTRODUCTION: Xeroderma pigmentosum is a relatively frequent genodermatosis in North Africa. It is characterized by abnormal sensitivity to ultraviolet light, responsible for the early occurrence of multiple cutaneous neoplasms. We present the results of the clinical and biological investigations in a family in which all its members exhibited xeroderma pigmentosum. PATIENTS AND METHODS: Since 1962, the father, mother, the 5 children and the maternal uncle were all followed-up in the dermatology department in Tunis for a variant of xeroderma pigmentosum. Clinical (dermatological, neurological and ophthalmologic), biological, photobiological and molecular biology investigations were carried out. RESULTS: Diagnosis of a variant of xeroderma pigmentosum was established on the delayed appearance (after the age of 4) of poikiloderma and the early onset of multiple carcinomas, without neurological disorders. Fifty-eight squamous cell and 3 basal cell carcinomas were diagnosed and treated by surgical exeresis or radiotherapy. The third child, treated with etretinate for 6 years, had developed 38 carcinomas. Contrary to the parents, whose first carcinomas had appeared at the age of 34 and 40 years, the cutaneous cancers in the children appeared early, between the ages of 17 and 24. The minimal erythematous dose was normal in all these patients. Conversely, the phototest revealed persistent erythema and the delayed appearance of multiple dyskeratosis cells. Molecular biology confirmed the diagnosis of xeroderma pigmentosum with the presence of a low level DNA repair. The third child, the father and the uncle respectively exhibited DNA repair rates of 32, 57 and 72%, compared with normal controls. The results of the complementarity tests conducted in the third child suggested that this family belonged to the genetic F group. Discussion The clinical and molecular data confirmed the diagnosis of xeroderma pigmentosum in this family and their genetic F group profile. However, this family exhibited clinical (the cutaneous involvement was more severe in the children) and molecular heterogeneity and the level of DNA repair was high in comparison with the levels (between 12 and 15%) reported by Japanese authors in group F xeroderma pigmentosum. The third child exhibited 10-fold more carcinomas that his siblings. This high rate of carcinoma may be explained by excessive exposure to sun and/or the retinoid treatment, particularly since his DNA repair rate (32%) was relatively high compared with that of severe (0-5%) and moderate (5-15%) forms of the disease.

Follow-Up Studies↗

[Value of imaging in GAPO syndrome].

GAPO syndrome is a rare genetic disorder. The term GAPO is the acronym for the manifestations: Growth retardation, Alopecia, Pseudoanodontia and Optic atrophy. We report the case of a 12 year-old boy with GAPO syndrome. Physical examination was remarkable for: dilated scalp veins with two flaccid masses of the vertex and the right mastoid area that was pulsatile with an audible bruit. Brain magnetic resonance imaging (MRI), magnetic resonance angiography (MRA) and cerebral angiography showed very prominent cortical veins, hypoplasia of the left transverse sinus, agenesis of the left jugular vein and left sigmoid sinus with 2 enlarged emissary veins underlying the palpable scalp masses. We suggest that brain MRI and MRA should be performed in patients with GAPO syndrome to detect anomalies of the intracranial venous circulation.

Alopecia↗

[Melanoma in xeroderma pigmentosum: 12 cases].

BACKGROUND: Xeroderma pigmentosum is a rare genodermatosis, with a defect affecting recovery of ultraviolet-induced damages and characterized by a high rate of malignancies of the exposed skin areas. We studied melanoma features of patients with xeroderma pigmentosum. PATIENTS AND METHODS: A retrospective study of xeroderma pigmentosum patients admitted to the Charles Nicolle Hospital of Tunis between 1973 and 1998. RESULTS: Two hundred sixteen patients with xeroderma pigmentosum were registered. Melanoma was present in 12 patients, 7 females and 5 males. Two patients were sisters. Cutaneous melanoma was found in 8 patients. Four patients presented with metastatic melanoma. The median age for development of the first melanoma was 17.5 years. All of the cutaneous melanomas were found on the face. Lentigo malignant melanoma was reported in 3 cases. The tumors were treated with surgical excision. Except for a melanoma affecting the orbit, characterized by a fatal outcome, no metastases were detected at the different investigations. DISCUSSION: Melanoma occurs frequently in patients with xeroderma pigmentosum, it has been reported in 5.5 p. 100 of cases and 11.3 p. 100 of patients with cutaneous carcinoma. The age of onset was low: 17.5 years. It appeared later than the carcinoma. The location of cutaneous melanoma in face in xeroderma pigmentosum patients indicates that they were caused mainly by sunlight exposure. Lentigo malignant melanoma was the most frequent type. Prognosis is difficult to define owing to the large number of other cutaneous malignancies. Apart from one case of rapidly fatal orbital melanoma, we recorded long survivals even in cases of melanoma revealed by metastases.

Adolescent↗

[GAPO syndrome].

INTRODUCTION: The GAPO syndrome is a rare but distinct genetic disorder. GAPO is an acronym for the manifestation of Growth retardation, Alopecia, Pseudoanodontia and Optic atrophy. The syndrome was first reported in 1947; to date, 24 cases have been reported. We report the first Tunisian case. OBSERVATION: We studied a 12 year-old boy with GAPO syndrome which was associated with peculiar facial appearance, umbilical hernia, hemangiomatous plaques of the neck, depigmented maculae arranged in a splashed pattern located in the trunk and the right upper limb. He had a pulsated mass in the right mastoid area and a bruit was audible, he had a second flaccid mass of the vertex. These tumefactions correspond to very developed commissure veins. DISCUSSION: In addition to the classical manifestations of the GAPO syndrome, the patients have a strikingly characteristic facial appearance and may also have umbilical hernia, skin redundance and prominent dilatation of scalp veins. Our case had depigmented maculae suggestive of incontinentia pigmenti achromians. This has never been reported previously. The pathogenesis of this syndrome is unknown and inheritance is considered to be autosomal recessive.

Alopecia↗

[Bites from stealthy arthropods from here to there].

Stealthy arthropods perpetrate their insults to humans through three distinct mechanisms including bites, stings or simple contact with the skin. The present review is a summary of the bite effects of spiders and diptera.

Diagnosis, Differential↗

[Cutaneous leishmaniasis].

Cutaneous leishmaniasis in its various forms is a frequent disorder in some parts of the world. It can represent an exotic souvenir for the traveller. Four main types of the disease are recognized. They have different prognoses of evolution. The parasite species, the animal reservoir and the geoclimatic environment influence the nature of human leishmaniasis.

Climate↗

[Profile of bullous pemphigoid. A report of 47 cases].

We report forty-seven cases of bullous pemphigoid recorded in the dermatology department of Charles Nicolle hospital in Tunis during 16 years. In Tunisia, bullous pemphigoid is at the second rank of acquired autoimmune bullous skin diseases, after pemphigus. The profile of bullous pemphigoid in our series differ from that reported in the literature by the more young age (67.2 years) and the male predilection but don't present any clinical an epidemiological particularity. Three atypicals forms were observed: a vesicular form, a localized form and a infantile form. Systemic corticosteroids were choice treatment for our patients.

Adolescent↗

[Junctional epidermolysis bullosa: should circumcision be allowed?].

BACKGROUND: Junctional epidermolysis bullosa is an uncommon heterogeneous syndrome transmitted by recessive autosomal inheritance. In addition to the classical skin lesions, the syndrome may include mucosal involvement which further complicates treatment. A few cases with urinary tract lesions dominating long-term prognosis have been reported. CASE REPORT: An child born in 1987 had junctional epidermolysis bullosa associated with urethral stenosis. Skin lesions typical of generalized atrophic benign junctional epidermolysis bullosa were observed. Urinary tract involvement developed after ritual circumcision and led to stenosis of the urethral meatus. Endourethral dilatations were unsuccessful and led to complete stenosis of the urethra, requiring life-long indwelling suprapubic catheter. DISCUSSION: Due to the risk of urinary tract involvement, we discuss the risks involved with circumcision in certain forms of junctional epidermolysis bullosa.

Child↗

Collision of primary malignant neoplasms on the skin: the connection between malignant melanoma and basal cell carcinoma.

Several studies have reported the association of cutaneous malignant melanomas (MM) with carcinomas. Collision malignancies cases from our files were retrieved. Among a series of 78,000 primary cutaneous cancers, 11 were collision tumors of MM with basal cell carcinoma and 106 were basosquamous carcinomas while no association was found between MM and squamous cell carcinomas. It is concluded that coexisting and confluent malignancies of the skin might not always be a random event.

Adult↗

Keratosis lichenoides chronica: an unusual case.

An unusual severe case of keratosis lichenoides chronica is presented. In addition to the typical lichenoid reticulated lesions, the patient had a prominent erythematosquamous involvement of the face and lips. Sclerodactylia was present and represented a yet undescribed manifestation of the disease.

Adult↗

[Comparative epidemiology of pemphigus in Tunisia and France. Incidence of foliaceus pemphigus in young Tunisian women].

INTRODUCTION: Recent studies have suggested that pemphigus foliaceus is quite frequent in young Tunisian women. In order to confirm this hypothesis, we compared the incidence of pemphigus in general in Tunisia with that in the Ile-de-France region. METHOD: All new cases of pemphigus diagnosed during a 6-year period were reviewed in our dermatology and pathology laboratories. These cases were classed as pemphigus foliaceus or pemphigus vulgaris on the basis of histology reports. RESULTS: In France, the incidence was 1-7 new cases per million per year (95 p. 100 confidence interval 1.4-2.1). Pemphigus vulgaris was diagnosed in 73 p. 100 of the cases with an incidence increasing with age. Sex ratio (F/M) was 1.2. The incidence observed in Tunisia was significantly higher than that observed in France with 6.7 new cases per million per year (95 p. 100 confidence interval 5.8-7.7). Pemphigus foliaceus was more frequent (61 p. 100), the sex ratio (F/M) was 4.1. Incidence was higher in young women, with 20 new cases of pemphigus foliaceus per million per year among women from 25 to 34 years of age. These levels were higher in rural desert areas. No familial cases were observed and only one case occurred in a child. DISCUSSION: These findings confirm the specific epidemiology of pemphigus in Tunisia, which appears to be similar and also different from that in Brazilian pemphigus. As in Brazil, there was a predominance of pemphigus foliaceus in young adults living in rural areas in poor socioeconomic conditions. However in Tunisia the disease predominates significantly in women and there are no familial and rare juvenile cases.

Adult↗