[Dermatofibrosarcoma protuberans: a study of 18 cases]

Dermatofibrosarcoma protuberans is the most common mesenchymatous skin tumor. It is often diagnosed late because of its slow development and the lack of symptoms. To elucidate the epidemio-clinical characteristics of dermatofibrosarcoma in our study. We report a retrospective study of 18 cases of DFS between 1989 and 2009. Mean age at diagnosis was 45.2 years. The average delay before consultation was 45.7 months. Three patients reported a history of trauma. The tumor was mainly located on the trunk or the back. The diagnosis of dermatofibrosarcoma protuberans was histologically confirmed in all cases. Immunohistochemical study was achieved in 6 cases and showed positive staining for CD34. The treatment consisted of surgical excision in all patients. Dermatofibrosarcoma protuberans is a low-malignancy potential skin tumor. Treatment of choice is surgery. The main risk is tumor relapse

[Hyalinosis cutis et mucosae: 5 cases from Tabarka [Tunisia]]

Hyalinosis cutis et mucosae [HCM], is a rare autosomal recessive genodermatosis. Cutaneous features are characteristic and allow to suspect diagnosis. To report a series of HCM. A retrospective study of all cases of HCM, diagnosed in a dermatology department over a period of 25 years [1983-2007]. Over the considered period, 5 new cases of HCM were diagnosed. Patients were aged between 14 and 41 years. They were 3 females and 2 males. All patients were native of Tabarka [northwestern Tunisia]. The age of the onset of the disease varied from neonatal period to 5 years. Hoarseness was the first clinical manifestation in all cases. Skin lesions developed between the ages of 3 and 8 years. Vesiculobullous lesions were observed in 2 patients. Moniliform blepharosis was seen in all patients. Warty and hyperkeratotic papules were observed in 3 patients. Diffuse thickening of the skin was seen in 3 patients. Lesions were primarily distributed on the face. All patients presented diffuse scars. Linear palmoplantar keratoderma was seen in one patient. Asymptomatic endocranial calcifications were noted in 4 patients. A pituitary adenoma was noted in one patient. Histopathological examination of a skin lesion revealed a typical pattern of HCM. Two patients were treated with systemic retinoids without improvement. We reported five new cases of HCM. All patients were native from Tabarka. We report also one case of linear palmar keratoderma associated with HCM. This association was not reported in the literature. Finally, the association HCM-pituitary adenoma, seen in one patient, may be fortuitous

[Cutaneous schwannoma: study of 26 cases]

Schwannoma is generally a benign tumour developing from Schwann cells. To study epidemiological, clinical and pathological features of scwannoma. Retrospective study about 26 cases of cutaneous schwannoma collected in our department over 24 years. Our patients were 12 females and 14 males. Mean age was 30 years. Clinically, schwannoma was a subcutaneous tumour which was painful in 11 cases. Individual tumours were located on the limbs [10 cases], on the head [9 cases] and the trunk [5 cases]. Multiple tumours were seen in two patients. Histopathological examination confirmed the diagnosis of schwannoma in all cases. Treatment was complete surgical excision in all cases. Cutaneous location of schwannoma is not rare. Usually, and contrarily to our series, there is no sex predominance. Clinically, schwannoma is located on the limbs and head. The diagnosis is confirmed by histopathological examination. Malignant epitheloid schwannoma diagnosed in one of our patients is a rare tumour. Schwannomas usually grow slowly and are poorly invasive. Malignant degeneration is exceptional. Treatment is complete surgical excision