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M Mokni

Publications and source records attributed to M Mokni.

At least 37 records · Page 2Linked to original sources
PubMed2004

[Extraskeletal osteosarcoma of the forearm: a case report].

Extraskeletal osteosarcoma is a rare soft tissue tumor. We report an exceptional case located in the forearm. A 62-year-old woman consulted for a tumor of the right forearm which she had noticed for six months. Physical examination revealed a 10 x 12 cm tumor with an ulcerated center. MRI demonstrated a heterogeneous mass exhibiting no connection with the bone or subjacent periosteum. Wide surgical resection was performed. The pathology study of the operative specimen confirmed the diagnosis of soft tissue osteosarcoma. The patient was given postoperative chemotherapy and was free of local recurrence or metastasis eighteen months after surgery. We discuss the present case and review data reported in the literature.

Female↗
PubMed2004

[Adenocarcinoma of the umbilicus].

INTRODUCTION: Most of the umbilical neoplastic processes are metastatic whereas primary adenocarcinoma is scarce and hard to distinguish from a secondary process. Our observation illustrates this difficulty. OBSERVATION: A fifty year-old woman had developed chronic omphalitis over several years. She consulted for a multinodular, erythemato-violine, oozing and deep infiltrating tumor of the umbilicus which had been developing for two months. The anatomopathological exploration of a biopsy of the lesion suggested a clearly differentiated adenocarcinoma whose digestive origin was the most likely. Investigations for a primary neoplasm were negative. Polychemotherapy was efficient with eight months recline. DISCUSSION: Umbilical metastases are referred to as Sister Marie Joseph nodule. They essentially correspond to a metastatic localization of an intra abdominal adenocarcinoma. The major problem consists in distinguishing a Sister Marie Joseph nodule where the neoplasm remains indefinite from an adenocarcinoma developed from an embryonic remainder. The existence in our patient's case history of a chronic omphalitis developing for Years and the negative tests for a primary neoplasm suggest a primary adenocarcinoma of the umbilicus.

Abdominal Neoplasms↗
PubMed2004

[Pemphigoid gestationis: a study of 20 cases].

INTRODUCTION: Pemphigoid gestationis is a bullous autoimmune sub-epidermal dermatosis, occurring during pregnancy and/or postpartum. The objective of our study was to define the epidemio-clinical profile, the histopathological and immunopathological features, the treatment and the course of pemphigoid gestationis in Tunisian patients. PATIENTS AND METHODS: This was a retrospective study concerning the cases of pemphigoid gestationis recorded between 1989 and 2003 in the dermatology department in La Rabta Hospital in Tunis. The patients were included according to clinical, histopathological and immunopathological criteria. RESULTS: We retained 20 patients: 15 multiparae and 5 primiparae. The average age at onset was 29. The first clinical signs appeared in the 3rd trimester in 60 p. 100 of all cases. Clinically, the urticarial patches were noticed in 90 p. 100 of all cases and blisters in 65 p. 100 of the cases. The eruption was located mainly on the trunk and the limbs. The face was affected in 7 cases, the mucous membranes in 3 cases, the palms in 2 cases and the soles in 1 case. Cutaneous histologic examination revealed a sub-epidermal blister in 11 cases and a lymphohistiocyte infiltrate in all cases. Direct immunofluorescence showed a linear deposition of the third component of the complement along the basement membrane zone in all cases. Fetal prognosis was assessed in 13 cases: 1 fetal death, 1 still-born, 3 miscarriages, 1 anencephaly and 2 cases of transit bullous affection in the new-born. DISCUSSION: Our results are similar to those in the literature, but with some particular aspects: the late onset of the pemphigoid gestationis in the course of the 3rd trimester of pregnancy, the frequent involvement of the face and the mucous membranes and the absence of the two main fetal risks: prematurity and hypotrophy. Moreover, we underline the efficiency of topical class I corticosteroids in the treatment of pemphigoid gestationis.

Administration, Topical↗
PubMed2004

[Lymphoepithelioma-like carcinoma of the uterine cervix: case report].

We report a case of a 79-year-old Tunisian woman who presented with post-coital bleeding. A polypoïd mass of the cervix was discovered. Histopathological examination of the specimen biopsy showed a squamous-cell carcinoma. The patient underwent radical resection. The definitive diagnosis of lymphoepithelioma-like carcinoma of the cervix was retained after histopathological and immunohistochemical examination. There was no local involvement and no metastasis. The lymphoepitheloma-like carcinoma of the uterine cervix is a rare entity with distinct morphological features and a usually good prognosis.

Aged↗
PubMed2004

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↗
PubMed2004

[Cutaneous blastomycosis: description of two cases in Tunisia].

Blastomycosis is an uncommon mycotic infection in Africa. Isolated cutaneous disease is extremely rare. The purpose of this report is to describe 2 cases of cutaneous blastomycosis. The first case Involved a 70-year-old rural woman who presented papulonodules associated with scars on the upper extremities and right leg, ongoing for 2 months. The second patient was a 47-year-old woman who presented 2 vegetating plaque areas above the left knee and on the left shoulder, ongoing for 4 months. In addition a bow-shaped scar resulting from a dog bite was observed. Histologic examination and culture on Sabouraud's medium and brain heart agar confirmed diagnosis of cutaneous blastomycosis in both patients. No visceral involvement was found. Blastomycosis is uncommon in Tunisia with only 6 previously published cases. The two cases reported here are interesting because involvement was confined to the skin suggesting cutaneous inoculation of Blastomyces dermatitidis.

Aged↗
PubMed2004

[Massive ovarian oedema].

Massive ovarian oedema is a rare tumor-like condition predominantly found in young women. It is considered malignant until otherwise proven because of its solid nature. The pre-operative diagnosis is very difficult. The frozen section examination can help for diagnosis and ensures conservative treatment with ovarian preservation. The authors report a case of a 23-years-old woman with right ovarian mass findings on ultrasound imaging. The diagnosis of massive ovarian oedema was made on frozen section after a right oophorectomy. Although most of reported cases has been handled by oophorectomy, the conservative treatment must be the ruler, especially since the disorder is benign and reaches the youth.

Adult↗
PubMed2003

A novel missense mutation in the gene encoding SLURP-1 in patients with Mal de Meleda from northern Tunisia.

BACKGROUND: Mal de Meleda (MDM) is a rare autosomal recessive skin disorder which belongs to the clinically and genetically heterogeneous group of palmoplantar keratodermas (PPK). Clinically, MDM is characterized by erythema and hyperkeratosis of the palms and soles with sharp demarcation that appears soon after birth and progressively extends to the dorsal surface of the hands and feet. OBJECTIVES: Except for the molecular study reported in Algerian families, MDM has not yet been investigated in the Maghrebian population, characterized by its heterogeneous ethnic background and a high rate of consanguinity. In this study we report genetic and molecular investigations of eight unrelated consanguineous Tunisian families including 17 affected individuals. METHODS: Eight large consanguineous MDM families who originated from cities of northern Tunisia, with a total of 17 patients and 22 unaffected family members were investigated. Families were genotyped with the following microsatellite markers: CNG003, D8S1751 and D8S1836. Mutation analyses were performed in affected patients, in both parents and in unaffected individuals. Linkage analysis was also performed. RESULTS: All the clinical features of MDM were constantly present. Nevertheless variable severity was noted among patients. Histological details were recorded. The haplotype analysis of markers CNG003, D8S1751 and D8S1836 revealed that all affected offspring were homozygous by descent for the three polymorphic markers. The maximum lod score value, 3.22, confirmed the evidence for linkage to the ARS gene. Three haplotypes were observed, and the findings suggest that at least three different mutations within the ARS gene segregate with these haplotypes. Three different mutations were identified, the 82delT mutation previously described and two novel missense mutations. CONCLUSIONS: The results suggest that the ARS gene is likely to be responsible for MDM in the eight Tunisian families. The clinical variability in the expression of PPK in MDM Tunisian patients might be accounted for by the intervention of modifier genes influencing the MDM phenotype.

Antigens, Ly↗
PubMed2003

[Importance of amastigote forms morphology to differentiate Leishmania infantum and Leishmania major species].

The microscopic study of the dermal smears of 62 cases of cutaneous leishmaniose, 27 infected by Leishmania (L.) infantum and 35 by L. major, showed that the amastigotes of L. infantum are meaningfully smaller (p < 0.001). This criteria is a simple pary alternative to distinguish these 2 species which have completely different epidemiology, recovery delay and prophylactic dispositions.

Animals↗
PubMed2002

Pemphigus is not associated with allotypic markers of immunoglobulin kappa.

The kappa light chain constant region of immunoglobulins bears polymorphic markers involved in susceptibility to various autoimmune diseases. To determine whether it also contributes to the occurrence of pemphigus, a group of autoimmune blistering skin diseases owing to pathogenic autoantibodies, the genotypic frequencies of Km allotypes were evaluated in patients with pemphigus foliaceus or pemphigus vulgaris and ethnically-matched healthy controls in both Tunisia and France. No difference in the distribution of Km genotype or allele frequencies was observed between patients and controls in either countries. Therefore, Km allotypes do not appear to constitute a genetic factor contributing to pemphigus.

Adult↗
PubMed2002

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↗
PubMed2002

[Bullous amyloidosis].

INTRODUCTION: The occurrence of skin damage during systemic amyloidosis is common, but the appearance of bullous lesions is rare. Only twenty-seven cases have been reported in the literature. We report our observation of bullous amyloidosis during progression of renal amyloidosis. OBSERVATION: A 61 year-old man, presented with white, soft, palpebral edemas of the lower limbs, without scutulum involvement, associated with a large cubital nerve that had appeared in March 1997. Biological explorations revealed a nephrotic syndrome. Pathologic study of the renal biopsy concluded in amyloidosis. Treatment with colchinine stabilized the renal damage. One year later, a non-pruriginous, papular and bullous eruption occurred, localized essentially in the axillary and inguinal-crural folds of the forearms and legs. In the presence of an amyloidal deposit and intra-epidermal detachment, the cutaneous biopsy was evocative of bullous amyloidosis. The search for concomitant myeloma was negative. Treatment with colchinine was effective. The bullous lesions disappeared after 2 months, and 21 months later, renal damage was still stable. DISCUSSION: These particularities in evolution are exceptional and have never been described. A hypothetical modification in the physico-chemical properties of the amyloidal protein might explain the bullous eruption and stabilization of renal damage.

Amyloidosis↗
PubMed2002

[Diffuse pseudotumorous oncocytosis of the parotid gland. A case report].

Pseudotumorous oncocytosis of the parotid gland is very uncommon and frequently misdiagnosed since it generally presents as a true tumor. The clinical presentation and imaging features of a 6-cm diameter left parotid mass led to the diagnosis of a tumor in a 73-year-old man. At pathology examination of the partial parotidectomy specimen the "tumor" was found to be a metaplastic oncocytic lesion. We discuss the diagnostic criteria, differential diagnosis, and etiopathogeny of this lesion.

Adenoma, Oxyphilic↗
PubMed2002

[Multiple keratoacanthoma centrifugum marginatum].

INTRODUCTION: Keratoacanthoma centrifugum marginatum is a rare variety of commonly isolated keratoacanthoma. The size of the lesions and prolonged evolution often raise therapeutic problems. CASE REPORT: A 63-year-old man presented with multiple keratoacanthoma centrifugum marginatum evolving for 6 months and predominating on the lower limbs. Because of the multiplicity and size of the lesions, the patient was treated at acitretine at the dose of 1 mg/kg/day for five months, leading to the regression of all the lesions. DISCUSSION: Keratoacanthoma centrifugum marginatum has rarely been reported in the literature. Our observation remains exceptional in the multiplicity and size of the lesions. The efficacy of retinoids, previously reported in the treatment of solitary giant keratoacanthoma, multiple keratoacanthoma and keratoacanthoma centrifugum marginatum, was demonstrated even in this handicapping form of keratoacanthoma.

Humans↗
PubMed2001

Management of pyoderma gangrenosum.

Management of pyoderma gangrenosum continues to be a therapeutic challenge, both because the low incidence of the disease makes it difficult to conduct large-scale randomized studies and because pathogenic mechanisms are not yet well defined. The selection of drugs and dosing schedules is therefore mainly guided by clinical experience.

Cromolyn Sodium↗