[Epdemioclinical aspects of dermatomyositis in the region of Tunis]

Dermatomyositis [DM] is a rare but serious disease. The aim was to evaluate the epidemiology, presenting clinical manifestations, therapeutic features and outcome of patients with DM. From January 1986 to December 2003, we collected retrospectively cases of DM identified at the dermatology department of Habib Thameur hospital. We included only patients with definitive diagnosis of DM. We included 13 patients, mean aged 32.85 years: 9 adults and 4 children. We identified 9 cases of adult DM among them 2 cases of which were of paraneoplastic DM and 4 cases of juvenile DM among them 1 case of amyopathic DM and 1 case of DM associated with connective tissue disease. The evolution of DM followed the course of the neoplasm for the 2 patients with paraneoplastic DM. All patients received corticotherapy initially. Patients were folio wed-up for an average of 3 years [2 months to 10 years]. We found no serious repercussions on the growth of children with juvenile DM. DM is rare in Tunisia. In our study, its frequency is about 0.72 patients per year. Age of onset of DM is markedly smaller than the one reported in the relevant literature. Our 2 cases of DM associated with cancer are considered like really paraneoplastic. Juvenile DM seems to have a relatively good prognosis

Paraneoplastic digital necrosis: a case report

Digital necrosis is a frequent vascular disease. Its very often reflects arterial disorders of macro or mirocirculation. Paraneoplastic digital necrosis is rare, however.Report a new case of paraneoplastic digital necrosis. Our patient was a 62-year old diabetic female patient with a history of total thyroid ablation at 50 year old. She presented with bilateral digital necrosis of the second, third and fourth fingers. During her recovery, we have found a biological inflammatory syndrome and abdominal echography revealed a metastatic liver. Radiological investigations in search for the primitive tumour were negative. The patient was referred to a specialized center for a palliative chemotherapy. Etiologies of digital necrosis are various, mainly iatrogen, professional, toxic, atheroma or systemic disease. In case of no evident cause, explorations should be gone to detect underlying malignancies

[Urticarian vasculitis. a case report after sulfamethoxazole-trimethoprime ingestion]

Urticarian vasculitis [UV] is an anatomoclinical disorder characterized by the association of urticarian papules lasting for more than 24 hours with histological cutaneous vasculitis. Several etiologies are associated with the disease. We report the case of a 30-year-old woman with a-two-month history of urticarial purpuric and necrotic cutaneous lesions of the low limbs. These lesions occurred after oral sulfam‚thoxazole-trim‚thoprime ingestion, 2 weeks before the skin eruption. Histological examination revealed a leucocytoclasic vasculitis and direct immunofluorescence showed a perivascular IgG and C3 deposits. Immunological, allergological and radiological investigations were normal. The pharmacological enquiry confirmed the imputability of sulfam‚thoxazole-trim‚thoprime in the onset of skin eruption; Mild cutaneous improvement was observed after 3 months of treatment with prednisone 0,5 mg/Kg/day. Drug induced urticaria constitutes a rare etiology of UV. Sulfam‚thoxazole-trimethoprime imputability has not been reported before among the drug responsible for UV. Management of patients is difficult and improvement usually occurs after several months because of the resistance of the skin lesions

[Xeroderma pigmentosum: Cutaneous, ocular, and neurologic abnormalities in 49 Tunisian cases]

This is a retrospective study about 49 cases of xeroderma pigmentosum [XP]. The goal was to determine the clinicalfeatures of XP in Tunisia. Our study revealed the predominance of a moderate form. Malignant skin tumors showed early. Squamous cell carcinoma [SCC] remained the most frequent skin malignancy in all clinical forms. Neurological abnormalities were more frequent in the moderate form. Mental retardation and peripheral neuropathy were the most common signs

[Dermatofibrosarcoma protuberans of darier and ferranda study of 28 cases in adult]

Dermatofibrosarcoma protuberans of Darier and Ferrand is a mes‚nchymatous skin tumor of intermediate malignant potential, characterised by slow growth, local malignancy and a high rate of recurrence. It is a rare tumor, accounting for approximately 0.1% to 0.18% of all malignant skin tumors. Peak incidence of the disease is between 20 to 40 years of age. We report a retrospective study of 28 cases of dermatofibrosarcoma protuberans. We reviewed the clinical features, histopathological and immunohistochemical aspects of this tumor

Histiocytome fibreux angiomatoide: Une tumeur rare a malignite intermediaire

Angiomatoid fibrous histiocytoma is a rare humour affecting young adults. Unlike conventional malignant fibrous histiocytoma, it's extension is only local thus giving a good prognosis. We report the cases of a 9 years-old girl and a 1 6 years-old boy presenting respectively, with an axiliary tumour 5cm of diameter and a paravertebral subcutaneous tumour 1,5cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection

[Epidemiologic and clinical study of condyloma acuminata in a dermatologic department about 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