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M R Kamoun

Publications and source records attributed to M R Kamoun.

At least 19 recordsLinked to original sources

Anti-desmoglein 1 antibodies in Tunisian healthy subjects: arguments for the role of environmental factors in the occurrence of Tunisian pemphigus foliaceus.

Pemphigus foliaceus is an autoimmune blistering skin disease mediated by autoantibodies directed against desmoglein 1 and occurs as a sporadic form throughout the world, or as an endemic form called fogo selvagem in Brazil. Healthy subjects living in Brazilian endemic areas produce antidesmoglein 1 antibodies, suggesting the role of environmental factors in the initiation of the autoimmune response. Tunisia was described recently as an endemic area where the disease is characterized by its high rate among young people, especially women. An enzyme-linked immunosorbent assay using recombinant desmoglein 1 as antigen was used to detect antibodies against desmoglein 1 and calibrated with sera from 67 French healthy blood donors, 20 French pemphigus foliaceus patients and patients with other bullous skin diseases. When sera from 179 healthy Tunisian blood donors were tested, 31 (17%) were found positive. The desmoglein 1 binding activity of these 31 sera was confirmed in 10 cases by indirect immunofluorescence analysis and/or immunoblotting using human epidermal extract. Subclass analysis of antidesmoglein 1 antibodies showed that they were almost exclusively of the IgG2 subclass in positive normal sera and of IgG4 subclass in patients with PF. Thus, antibodies against desmoglein 1 are prevalent in normal subjects living in Tunisia which, along with their IgG2 isotype, suggests the role of the environment in the pathogenesis of this endemic type of pemphigus foliaceus and the need for additional factors to switch from a subclinical to a clinical form of the disease.

Adolescent↗

Intramuscular bipenicillin vs. intravenous penicillin in the treatment of erysipelas in adults: randomized controlled study.

The objective of the study was to evaluate the efficacy of intramuscular penicillin: mixture of benzyl penicillin and procain penicillin (2 MU x 2 times daily) and intravenous benzyl penicillin (4 MU x 6 times daily) in the treatment of hospitalized adult patients with erysipelas. A prospective randomized unicentric trial was conducted. In total, 112 patients entered the study; 57 in the intramuscular group and 55 patients in the intravenous group completed the trial. The failure rate was 14% for intramuscular group and 20% for the intravenous group (P = 0.40). Local complications such as of the leg abscesses were observed in the two groups (intravenous 9.1%, intramuscular 7%; P = 0477). Of the patients treated with intravenous benzyl penicillin, 25.5% presented complications related to the route (venitis). Intramuscular penicillin should be considered an effective and well-tolerated treatment of erysipelas in adult patients.

Adult↗

[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↗

Tunisian endemic pemphigus foliaceus is associated with desmoglein 1 gene polymorphism.

Desmoglein 1 is the target antigen and probably the initiating immunogen of the autoantibody response in pemphigus foliaceus (PF), a blistering autoimmune skin disease. We previously showed that the desmoglein 1 gene (DSG1) is polymorphic and that one of its variants is associated with the sporadic form of PF observed in France. Herewith, we report, based on a case-control analysis, that the same DSG1 polymorphism participates in susceptibility to the endemic form of PF seen in Tunisia and, thus, show that common genetic factors govern the breakage of tolerance to desmoglein 1 in different epidemiological and environmental situations.

Adolescent↗

[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↗

[Atopic dermatitis in Tunisia: epidemiological and clinical aspects].

INTRODUCTION: The prevalence of atopic dermatitis is usually high in western countries, varying from 18 p. 100 to 20 p. 100. Recent studies suggest an increasing of this frequency. The aim of our study is to determine the epidemiological and clinical characteristics of atopic dermatitis in Tunisia through the analysis of a retrospective cohort. PATIENTS AND METHODS: We retrospectively studied all the medical reports of atopic dermatitis registrated in the department of dermatology during a 7 years period (1992-1998). We analysed epidemiological and clinical features of every medical report. We used diagnosis criteria of Hanifin and Rajka. RESULTS: Four hundred fifty-one cases of atopic dermatitis have been diagnosed (54 adults and 397 infants). Relative frequency of new cases of atopic dermatitis compared to new diagnosis was 0.37 p. 100 in 1992 and 0.72 p. 100 in 1998. Mean age was 3.2 years. A personal history of atopy was observed for 15.2 p. 100 of patients and a family history of atopy for 35.9 p. 100. A few numbers of complications were observed. Bacterial infections interested 15.3 p. 100 of cases. Hospitalisation was needed for 4 patients (0.88 p. 100). Class III and IV topical corticosteroids were usually used (84.8 p. 100). Potent corticosteroids (class I) were required for only 3.2 p. 100 of cases. CONCLUSIONS: We notice a low frequency of atopic dermatitis in this study and a predominance of mild forms of the disease. Other studies are needed to confirm these results and to determine the prevalence of atopic dermatitis in Tunisia.

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↗

[Epidemiologic/clinical profile of condyloma acuminata in a dermatology service. Report of 232 cases].

The incidence of condylomata acuminata is increasing in all countries. They are one of the most frequent sexually transmitted disease (STD). The authors reported the experience of the department of Dermatology of charles Nicolle's hospital between the year 1979 and 1998. This HPV infections were in the third position of the STD and occurred mainly in young males. The clinical lesions are genital condylomata. They were successfully treated with electrodessication or cryotherapy.

Adult↗

[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↗

Immunoblot and immunoelectronmicroscopic analysis of endemic Tunisian pemphigus.

Tunisian pemphigus is a newly described form of endemic pemphigus whose clinical, histological and epidemiological characteristics have recently been detailed. The objective of this study was to analyse the binding properties of autoantibodies present in sera from patients with endemic Tunisian pemphigus using immunoblotting and indirect immunoelectron microscopy (IEM). Thirty patients with pemphigus foliaceus (PF) and six with pemphigus vulgaris (PV) seen in the dermatology department of Tunis Hospital between 1992 and 1994 were selected for this study. Seven of 30 (23%) and six of 12 (50%) PF sera tested bound to the 160 kDa band of desmoglein 1 when tested on bovine tongue and human epidermal extracts, respectively. Two of six and two of three PV sera tested bound to the 130 kDa desmoglein 3 in these two extracts. Immunoblot and indirect IEM showed that 24 of 30 (80%) PF sera contained IgG1, IgG3 or IgG4 antibodies that bound to a 185-kDa polypeptide localized on the desmosomal plaque. This immunological analysis showed that most endemic Tunisian pemphigus sera correspond to PF sera and are characterized by a high frequency of autoantibodies directed against a recently identified 185-kDa antigen of the desmosomal plaque.

Adult↗