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ABSTRACT: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma.
A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma.
Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features.
Annales de Dermatologie et de Vénéréologie 03/2013; 140(3):206-8. · 0.72 Impact Factor
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A Hawilo,
R Abdelmalek,
A Mebazaa,
O Addouni,
F Kanoun,
D El Euch,
S Haouet,
M Zitouna,
M Mokni, A Ben Osman,
T Ben Chaabane
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ABSTRACT: Tuberculosis of the breast (BT) is a rare extrapulmonary localization for tuberculosis that mainly affects young women during their child-bearing years. Objective: We report eight cases of BT and describe its clinical characteristics, outcomes, and differential diagnoses. Patients and methods: This retrospective study collected all cases of BT diagnosed at our hospital's infectious disease department in Tunisia from 2000 through 2009. We assessed the epidemiologic and clinical findings and reviewed the laboratory, histology, and radiology results, treatment data, and outcomes. Results: Eight women (mean age: 52.5 years, range: 31-75) had BT. Clinical features included retracted erythematous lesions in four cases, a fluctuating abscess in two, and a well-defined nodule in two. The biopsy culture was positive in two cases. Histology results showed a tuberculoid granuloma with caseous necrosis in all cases. Antituberculosis drugs were administered for 8 to 12 months. Two patients underwent surgery. Outcome included total regression of BT for all women. Conclusion: Clinical features of BT are often misleading and can easily be mistaken for those of breast cancer. Identification of the Koch bacillus or the presence of a tuberculoid granuloma with caseous necrosis on histology facilitates diagnosis. The treatment consists essentially of antituberculosis drugs. Despite its rarity, BT must not be misjudged, especially in countries where tuberculosis is endemic.
Medecine et sante tropicales. 11/2012;
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M Ammar,
C Bouchlaka-Souissi,
C A Helms,
I Zaraa,
C T Jordan,
H Anbunathan,
R Bouhaha,
S Kouidhi,
N Doss,
R Dhaoui, A Ben Osman,
A Ben Ammar El Gaied,
R Marrakchi,
M Mokni,
A M Bowcock
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ABSTRACT: Background: Psoriasis is a relapsing chronic inflammatory skin disease affecting all populations groups with a peak prevalence of 3% in northern European and Scandinavian Caucasians. Epidemiologic studies have implicated a genetic component to psoriasis. In the last 12 years multiple genome-wide linkage analyses have identified putative susceptibility loci on several chromosomes, with a major locus in the MHC region. Objectives: In order to investigate the genetic basis of familial psoriasis in Tunisian population we undertook a genome-wide linkage scan in seven multiplex psoriatic families from Tunisia. Methods: Following SNP genotyping on the Affymetrix 10K SNP array, we performed non-parametric linkage (NPL) multipoint analyses to identify genotypes and obtain evidence for linkage with psoriasis across the genome. Results: No chromosomal region gave consistent evidence for linkage, providing evidence for genetic heterogeneity in Tunisian psoriasis families. Significant evidence for linkage of psoriasis to chromosome 2p12 was seen in one family. We also identified several regions of tentative psoriasis linkage on chromosomes 2q, 4q, 6p, 11q, 12q, 9q and 13q. One family exhibiting suggestive evidence for linkage to 17q25 (PSORS2) was identified and all affected members harbored a p.Gly117Ser mutation in CARD14 (caspase recruitment domain family, member 14), recently described to lead to psoriasis in a large family from the U.S. Conclusions: Our results support the genetic heterogeneity of psoriasis in the Tunisian population, provide confirmatory evidence for a novel psoriasis locus at chromosome 2p12 and reveal a psoriasis family with a mutation at PSORS2.
British Journal of Dermatology 09/2012; · 3.67 Impact Factor
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ABSTRACT: Acute generalized exanthematous pustulosis (AGEP) is an uncommon pustular eruption characterized by small nonfollicular pustules on an erythematous background, sometimes associated with fever and neutrophilia. Over 90% of cases are drug-induced; however, it can be caused in rare cases by other agents. We report two cases of AGEP secondary to ingestion of Pistacia lentiscus essential oil, the first two such cases to our knowledge. The cutaneous morphology, disease course and histological findings were consistent with a definite diagnosis of AGEP, based on the criteria of the EuroSCAR study group. These two cases highlight the need to consider herbal extracts as a potential rare cause of AGEP and to ensure the safety of herbal medicines.
Clinical and Experimental Dermatology 06/2012; 37(4):361-3. · 1.20 Impact Factor
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M Ben Ahmed,
I Zaraa,
R Rekik,
A Elbeldi-Ferchiou,
N Kourda,
N Belhadj Hmida,
M Abdeladhim,
O Karoui, A Ben Osman,
M Mokni,
H Louzir
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ABSTRACT: Auto-reactive cytotoxic T lymphocytes play a key role in the progressive loss or destruction of melanocytes in vitiligo but the mechanism underlying the loss of self-tolerance is unknown. A deregulation of regulatory T-cell biology has recently been suggested. The analysis of the suppressive effects of peripheral T regulatory cells in vitiligo patients revealed a functional defect in seven of 15 cases. This defect was strongly correlated with disease activity. The evaluation of the percentage of peripheral regulatory T lymphocytes did not reveal any intrinsic quantitative defect. Yet, a decrease in the percentage of such cells was noted in patients with progressive forms, suggesting a recruitment of regulatory T cells from the peripheral blood to the site of injury. This was further corroborated by the significant increase of Forkhead box P3 expression in the vitiliginous skin of patients. Our data support the involvement of a functional defect of peripheral regulatory T cells in the pathogenesis of vitiligo and open new possibilities to advance therapeutic approaches.
Pigment Cell & Melanoma Research 01/2012; 25(1):99-109. · 5.06 Impact Factor
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ABSTRACT: Dowling-Degos disease is a rare and benign inherited dermatosis.
A 53-year-old woman presented with generalized histologically confirmed Dowling-Degos disease revealed 8 years after psoralen photochemotherapy (PUVA) for psoriasis. This presentation was special in terms of its considerable spread as well as the absence of comedone-like and punctate scars.
Dowling-Degos disease is a reticulate pigmentary disorder of the flexures associating prominent comedone-like lesions and pitted scars. Diagnosis is based on clinical and histopathological examination, which allows this entity to be differentiated from other reticulate pigmentary disorders. A literature review failed to provide any indication that PUVA therapy either aggravates or reveals Dowling-Degos disease, a finding which we feel merits mention.
Annales de Dermatologie et de Vénéréologie 01/2012; 139(1):54-7. · 0.72 Impact Factor
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I Zaraa,
S Trojjet,
F Ishak,
J Cherif,
H Azouz,
B Louzir,
D El Euch,
M Mokni,
N Kchir,
M Zitouna,
M Beji, A Ben Osman
Médecine tropicale: revue du Corps de santé colonial 02/2011; 71(1):16.
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ABSTRACT: Transient neonatal pustular melanosis is a common, benign, but little known dermatosis in newborns. Diagnosis of transient neonatal pustular melanosis is made clinically, by the presence of vesiculopustular and pigmented macular skin lesions. This benign spontaneously regressive dermatosis should be distinguished from several serious infectious neonatal diseases. We report a case of transient neonatal pustular melanosis and discuss the nosologic problems and differential diagnosis of this entity.
Archives de Pédiatrie 01/2011; 18(3):291-3. · 0.30 Impact Factor
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ABSTRACT: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disease of undetermined origin. It is characterized by the presence of nodular pseudo-tumors corresponding microscopically to a vascular proliferation within an inflammatory infiltrate made up of lymphocytes, macrophages, and eosinophils. The authors describe 7 cases of ALHE.
The 7 cases were diagnosed over a period of 19 years (1990-2008). Clinical data and histological slides were brought from the departments of dermatology and pathology of the Rabta Hospital.
The 7 patients were 4 women and 3 men with an average age of 34.5 years. The cephalic localization was the most frequent. Lesions were solitary or multiple and formed papules or plaques of variable color. The diagnosis was based in all cases on histological findings.
The main disease in the differential diagnosis of ALHE is Kimura disease, but the 2 entities have several clinical and histological differences. The pathogenesis remains unclear and there is no consensus on the best treatment.
Dermatology online journal 01/2011; 17(2):1.
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A Mebazaa,
M El Asmi,
W Zidi,
Y Zayani,
R Cheikh Rouhou,
S El Ounifi,
F Kanoun,
M Mokni, A Ben Osman,
M Feki,
H Slimane,
N Kaabachi
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ABSTRACT: Background A significant association between psoriasis and the metabolic syndrome (MetS) has been frequently reported.Objective The aim of this study was to specify the main factors that determine the MetS in psoriatic Tunisian patients.Methods A case–control study has included 164 psoriatic patients and 216 controls.Results The prevalence of MetS was higher in cases than in controls but without statistical differences [35.5% vs. 30.8%, odds ratio (OR): 1.39 CI: 0.88–2.18; P = 0.095]. According to gender, the prevalence of MetS was significantly increased only in psoriatic women (47.4% vs. 30%, OR: 1.89, CI: 1.11–3.21; P = 0.01). A multiple logistic regression, considering the effect of age, and gender, showed that the prevalence of MetS was significantly higher in cases than in controls (OR: 1.73, CI: 1.06–2.82; P = 0.03). MetS components analysed seperately showed a significantly higher prevalence of decreased high-density lipoprotein cholesterol (HDLc) (60.9% vs. 35.9%, OR: 2.77, CI: 1.8–4.27, P < 0.001) and for increased hypertension (50% vs. 40%, OR: 1.48, CI: 0.97–2.257, P = 0.04) in psoriatic patients. According to gender, HDLc was significantly decreased in both genders (male: OR: 2.075, CI: 1.24–3.47, P = 0.004; female: OR: 3.58, CI: 2.07–6.19, P < 0.0001), while hypertension was increased only in psoriatic men (OR: 2.09, CI: 1.24–3.51, P = 0.004) and abdominal obesity only in psoriatic women (OR: 2.31, CI: 1.30–4.11, P = 0.002).Conclusion Decreased HDLc is the main biological abnormality that characterized MetS in Tunisian psoriatic patients. Moreover, contrary to men, psoriatic women have shown a significantly higher prevalence of MetS, which is, in addition to decreased HDLc, mainly attributed to abdominal obesity.
Journal of the European Academy of Dermatology and Venereology 10/2010; 25(6):705 - 709. · 2.98 Impact Factor
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ABSTRACT: Dermatofibrosarcoma protuberans (DFSP) is a relatively rare skin neoplasm. Usually affecting adults, the incidence in children is even less frequent. Through a report of three pediatric cases of DFSP, we describe the particularities of this tumor in children. Three boys aged 8, 9, and 15 years presented with a firm nodular skin lesion of the trunk, varying in size from 1 to 5 cm. No previous trauma event had occurred. Diagnosis was confirmed in all cases by immunohistological study. Surgical excision was performed in all cases. No recurrence was evident during the follow-up period of, respectively, 15, 36, and 49 months. The DFSP is an infiltrative tumor of intermediate malignancy, with a limited potential for metastasis (<5%) but a high rate of local recurrence (≥ 50%). The incidence in children is even less frequent. In children, its seemingly benign clinical appearance may explain delays in diagnosis; the majority of lesions affect the extremities, suggesting a potential role-played by injury. In our observations, however, as in adults, the trunk was the site of occurrence. Despite the uncertain pathogenesis of this tumor, the finding of certain characteristic histopathological features helps establish an accurate diagnosis. As in adults, surgical treatment with large surgical margins remains the best practice for children with DFSP, directly affecting the prognosis.
Archives de Pédiatrie 10/2010; 18(1):23-7. · 0.30 Impact Factor
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A Mebazaa,
D El Euch,
R Abdelmalek,
H Azouz,
S Trojjet,
H Zribi,
R CheikhRouhou,
I Zaraa,
M Zitouna,
T Ben Chaabane,
M Mokni, A Ben Osman
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ABSTRACT: The purpose of this report is to describe a case involving Kaposi sarcoma as the inaugural manifestation of HIV infection at the full-blown AIDS stage. The patient was a 59-year-old Tunisian man who presented with profuse subcutaneous nodules and multiple lymph nodes. Treatment was based on antiretroviral therapy in association with radiotherapy.
Médecine tropicale: revue du Corps de santé colonial 08/2010; 70(4):403-5.
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International journal of dermatology 06/2010; 49(6):715-6. · 1.18 Impact Factor
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ABSTRACT: Mycetoma is a chronic infection of cutaneous and subcutaneous tissue that can be caused by fungi or bacteria. It is endemic in tropical and subtropical areas but rare in Tunisia.
The purpose of this report is to describe epidemioclinical features, treatment and outcomes in patients presenting mycetoma in Tunisia.
The files of all patients treated for mycetoma in the Dermatology Department of La Rabta Hospital in Tunisia from 1982 to 2006 were retrospectively reviewed.
A total of 15 cases of mycetoma were recorded during the study period. There were 6 men and 9 women with a mean age of 53.2 years. The most common clinical presentation was infiltrated erythematous plaques with sinus tracts (fistulas). Lesions were located on the foot in 12 cases. Thirteen patients reported the presence of grains in fluid discharging from fistulas. The cause of mycetoma was actinomycetes, i.e., Actinomadura madurae, in 9 cases and fungus in 6 cases including 3 due to Madurella mycetomi and 2 to Pseudallesheria boydii. Treatment was based on oral antibiotics for actinomycetoma and oral antifungals for eumycetoma.
Mycetoma in Tunisia is still uncommon with a slight female predominance. The foot is the most frequent location. Diagnosis can be accomplished by direct mycologic examination, culture, and histololgy. There is no consensus on treatment that is often prolonged with numerous relapses.
Médecine tropicale: revue du Corps de santé colonial 06/2010; 70(3):269-73.
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Annales de Dermatologie et de Vénéréologie 05/2010; 137(5):391-2. · 0.72 Impact Factor
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R Benmously-Mlika,
M Bchetnia,
S Deghais,
S A Ben Brick,
C Charfeddine,
A Debbiche,
S Haouet,
M Mokni,
S Abdelhak,
M R Kamoun, A Ben Osman,
S Fenniche,
I Mokhtar
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ABSTRACT: Most of the published reports on Hailey-Hailey disease (HHD) come from European and Asian countries. We report here the clinical and genetic investigation of 20 patients affected with HHD in Tunisia.
Affected individuals from three large teaching hospitals in Tunis were recruited for the study over a 25-year period. Nine patients were identified through the active files and examined together with their family members that were visited in their respective regions. We have clinically examined in total 65 individuals and then identified 11 new cases. Patients were included on the basis of evocative skin lesions, biopsy proven HHD and negative immunofluorescence. Investigations to rule out fungal, bacterial and viral infections were done according to clinical symptoms.
Twenty patients (12 males and 8 females) from 8 families were included in the present study with more than 55% that were undiagnosed before this investigation. Four patients had mild disease, eight had moderate disease and another eight had severe disease, among whom seven were females. Parental consanguinity was found in 7 cases out of 20 cases (35%). The neck region was first affected in half (4/8) of the male patients. Groins were first affected in 42% (5/12) of the female patients. Depression complicated the course of the disease in two female patients with severe HHD. We report an original association of supernumerary nipples with HHD in two sisters from the north of Tunisia. In 10 patients, the disease has become less troublesome with aging.
HHD is underestimated. Physicians must be aware of this disease in case of resistant intertriginous dermatosis especially with a positive family history as nine out of 20 patients were misdiagnosed.
International journal of dermatology 04/2010; 49(4):396-401. · 1.18 Impact Factor
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ABSTRACT: Chromoblastomycosis is a chronic subcutaneous fungal infection caused by dematiaceous saprophytic moulds. We report a case of chromoblastomycosis due to Fonsecaea pedrosoi observed in man from the Baja region of Tunis. He presented since one year an erythemato-squamous atrophic plaque localised at the abdomen area. Clinical remission was obtained after cryotherapy and terbinafine.
Médecine tropicale: revue du Corps de santé colonial 02/2010; 70(1):81-3.
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Annales de Dermatologie et de Vénéréologie 01/2010; 137(1):84-5. · 0.72 Impact Factor
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ABSTRACT: Psoriasis is a chronic inflammatory skin disease. The familial nature of psoriasis has long been recognized. Aim of our study was to describe the epidemiological, clinical and genetic features of familial psoriasis.
Through a prospective study we investigated during a study period of lyear (2006-2007) 9 Tunisian unrelated multiplex families. Patients with psoriasis and their available family members were examined by the same dermatologist.
Thirty nine individual presented psoriasis (25 men and 4 women), with a mean age at onset about 19.8 years. With the systematic exam of member's family we discover 11 cases of unknown psoriasis. The common form of psoriasis was the preponderant one (37 cases). The nails, the scalp, the mucous membranes were involved respectively in 21, 12 and 13 cases. The psoriasis was severe in 11 cases.
Through this study we find similar epidemiological and clinical features of those reported previously. The intra and inter-familial variability was evident in our patients.
La Tunisie médicale 11/2009; 87(11):750-4.
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ABSTRACT: Darier's disease (OMIM 124200) is an autosomal-dominant skin disorder characterized by warty papules and plaques in seborreheic areas, palmo-plantar pits and distinctive nail abnormalities. The disease has complete penetrance in adults and variable expressivity. It is caused by mutations in the ATP2A2 gene, which encodes the sarco/endoplasmic reticulum Ca(2+) ATPase type 2 isoform (SERCA2).
We report histological investigations of six unrelated Tunisian families including 15 affected individuals with Darier's disease mutations.
The typical histological features of Darier's disease have been observed in the 15 patients. Variable histological features have been observed among Tunisian patients ranging from mild to moderate lesions of Darier's disease. A significant correlation has been observed between the clinical presentation of the Darier's disease (mild or moderate) and the intensity of the histological features. Isolated acral form of Darier's disease was seen in one case. Two distinct original associations have been observed: Darier's disease/pemphigus vulgaris in one patient and Darier's disease/ichtyosis in the other patient.
Our findings confirmed the clinical heterogeneity of Darier's disease on the basis of histological study. The intensity of the histological features could be closely correlated to the severity of Darier's disease clinical presentation.
Journal of the European Academy of Dermatology and Venereology 06/2009; 23(10):1178-83. · 2.98 Impact Factor
