[Autoimmune progesterone dermatitis]. / Dermatose auto-immune à la progestérone.

BACKGROUND: Autoimmune progesterone dermatitis (AIPD) is caused by an immune reaction to endogenous progesterone following exposure to exogenous progesterone, especially in oral contraceptives and intrauterine devices. Skin eruption develops cyclically during the luteal phase of the menstrual cycle when progesterone levels are elevated. The resultant clinical symptoms are frequently confused with other forms of dermatosis. We describe a young woman presenting AIPD. PATIENTS AND METHODS: A 23-year-old woman had a history of a chronic pruritic papular-vesicular eruption on the trunk, present since the onset of puberty and unsuccessfully treated with antihistamines and topical steroids. Skin lesions normally began a few days before menstruation and resolved a few days afterwards. Intradermal testing using progesterone was positive, thereby confirming the diagnosis. The patient's skin lesions improved under oral contraceptives designed to inhibit ovulation and there was no relapse during subsequent menstrual cycles. DISCUSSION: AIPD is a rare autoimmune disorder. The diagnostic criteria of AIPD include recurrent cyclical worsening of skin lesions and symptomatic improvement after inhibition of progesterone secretion by suppression of ovulation. The pathogenesis is unclear. Positive response to various tests with progesterone suggests involvement of various mechanisms such as antibodies and/or cell-mediated immune reactions. CONCLUSION: AIPD is a rare disease that can seriously affect patients' quality of life and which may even be life-threatening in certain cases.

[Pyoderma gangrenosum: 6 cases]. / Le pyoderma gangrenosum: à propos de 6 observations.

Pyoderma gangrenosum (PG) is an uncommon extensive cutaneous ulceration belonging to the neutrophilic disease spectrum. It is associated to a systemic disease in almost 50% of cases. The diagnosis primarily relies on clinico-pathological features and the treatment is empirical. We report a retrospective series of 6 observations of PG (4 women and 2 men, median age = 43 years) enrolled over 15 years. The diagnosis was established based on the presence of 2 major criteria and 2 minor criteria of the disease. In 3 patients, PG was associated to an already known ulcerative colitis. The treatment consisted in general corticotherapy.

[Dermatological problems of musicians: a prospective study in musical students]. / Dermatoses des musiciens: étude prospective chez des étudiants en musique.

INTRODUCTION: Musicians are at risk of developing particular skin conditions related to their activity. The purpose of our work was to determine the frequency, describe the clinical aspects and identify the risk factors of specific dermatological problems in musical students. POPULATION AND METHODS: We carried out a prospective epidemiological study between April 2003 and April 2004 among music students registered in four musical institutes of Tunisia. The musicians were questioned and examined in systematic fashion at their institutes. Demographic data, characteristics of instrumental practice and dermatologic manifestations were collected. Five hundred and ninety-four students were examined. The sex-ratio was 1.6 with a mean age of 22.0 +/- 4.0 years. The statistical analysis was performed using SPSS software for Windows (version 12.0). RESULTS: Two hundred and ninety-five students (49.7%) had at least one dermatologic lesion. In multivariate analysis, the following factors were significantly correlated with dermatological problems: male gender, age > or = 20 years, history of musical activity > or = 5 years, practice > or = 10 hours/week and percussion instruments. Nine types of lesions were determined: corns and calluses, pigmentation abnormalities, irritant contact dermatitis, mechanical acne, erythema, friction bulla, nail diseases, cicatricial alopecia and allergic contact dermatitis. Corns and calluses were significantly the most frequent (P<0.001). DISCUSSION: The frequency of dermatological lesions was high compared to data reported in the literature, 20 and 32% respectively in the studies by Onder and Rimmer. This difference could be due to the greater number of percussionists in our study.

[Leiomyosarcoma of the lip]. / Léiomyosarcome de la lèvre.

BACKGROUND: Superficial leiomyosarcomas are rare malignant tumors that may be subdivided into cutaneous and subcutaneous cases. CASE-REPORT: A 58 year-old man was seen for a lower lip tumor that appeared 3 years earlier. Histopathologic and immunohistochemical examinations showed features of cutaneous leiomyosarcoma. DISCUSSION: Cutaneous leiomyosarcoma may derive from the arrector pili, smooth muscle of sudoral glands or genital dartoic muscle. The subcutaneous form arises from smooth muscle wall of blood vessels. Superficial leiomyosarcomas occur frequently in the extensor surface of the lower extremities and involve the mucosal area in rare cases. Only five cases arising in the lips have been described. Lip cutaneous leiomyosarcoma may derive from ectopic sweat glands of the lips or from a hypodermic tumor that extends to the lip.

[Analysis of immunoglobulin heavy chain genes rearrangement by PCR from paraffin-embedded tissue in B-cell lymphomes in Tunisia]. / Analyse des réarrangements des gènes des chaînes lourdes des immunoglobulines par PCR sur tissu enrobé en paraffine: application aux lymphomes B en Tunisie.

OBJECTIVE: To study the lymphoid clonality on Tunisian B-cell lymphomas cases by polymerase chain reaction (PCR)-based techniques using DNA from paraffin-embedded tissues. MATERIAL AND METHODS: Here we conducted a retrospective PCR clonality study on 73 cases of B-cell lymphomas and 12 reactive lymphoid tissues. The quality of DNA extracted was tested by beta-globin PCR. Consensus primers directed at the FRIII-VH and FRII-VH regions of the immunoglobulin heavy chain (IgH) gene were used to detect clonality. RESULTS: The results showed that 52 of 73 (71%) B-cell lymphomas exhibited good quality of amplifiable DNA. Clonality was found in 77% of cases using the set of primers FRIIIa/LJH/VLJH and in 65.5% using the set of primers FRIIa/LJH/VLJH. Lymphomas derived from pregerminal centre showed a high rate detection of clonal IgH gene rearrangement (100%) compared to other group of tumors derived from germinal centre or postgerminal centre (74.5%). None of the polyclonal controls gave a clonal pattern. CONCLUSION: This is the first large series of PCR clonality study of IgH gene rearrangements on B-cell lymphoma from Tunisia. Our results were similar to other reports in terms of sensitivity and specificity of these techniques and confirm the interest of that PCR for detecting clonal IgH gene rearrangements in lymphoma.

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.