[Multinucleate cell angiohistiocytoma].
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Biomedical subjects
Publications and source records attributed to I Zaraa.
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BACKGROUND: Acrodermatitis enteropathica is a rare autosomal recessive disorder, caused by impaired absorption of zinc from the gastrointestinal tract. Symptoms of acrodermatitis enteropathica occur within the first few months after birth and tend to appear shortly after discontinuation of breast-feeding. We report a breast-fed infant with acrodermatitis enteropathica. CASE REPORT: A full term, 4-month-old girl, consulted in dermatologic department for persistent and refractory anogenital lesions since the age of 1 month, with progressive erythematous, vesiculous and squamous lesions, sometimes erosive in a peri orificial and acral pattern. She was calm and healthy baby. She was breast feeding. The diagnosis of acrodermatitis enteropathica was confirmed by decreased plasma zinc level (14 microg/100 ml). Breast milk zinc levels was low (46 microg/100 ml), as plasma zinc level of the mother (94 microg/100 ml). A genetic study showed that she was homozygous for the mutation, whereas her brother and parents were heterozygous. She was given zinc sulphate, and her condition has improved significantly. DISCUSSION: Acrodermatitis enteropathica is characterized by a characteristic clinical feature and the diagnosis is confirmed by decreased plasma zinc level. Acrodermatitis enteropathica in exclusively breast fed infant is rare, it was essentially reported in premature babies. Our case report is particular because it's concerning a full-term breast-fed infant, with zinc deficiency in breast milk and mother's decreased plasma zinc level.
BACKGROUND: Dyskeratosis follicularis (Darier's disease) is rare autosomal dominant disease characterized by the loss of adhesion between epidermal cells and by abnormal keratinization. METHODS: We performed a retrospective study of all the patients diagnosed with Darier's disease at the Department of Dermatology of Charles Nicolle Hospital of Tunis, between 1971 and 2002. RESULTS: During the observation period, we identified 12 patients with Darier's disease; five males and seven females with a mean age of 17.36 years. No family history was found in eight patients. Skin lesions in the form of keratotic papules were noted in seborrhoeic areas, essentially the face (nine patients), chest and scalp. Seven patients had nail lesions. UV light exposure exacerbated the disease symptoms in seven cases. The patients were treated with topical and systemic retinoids (six cases). CONCLUSION: Although Darier's disease has a chronic course, most patients manage to lead a relatively normal life. Treatment is usually unsatisfactory despite much progress in understanding of the underlying abnormalities in Darier's disease.
Dermatofibrosarcoma protuberans (DFSP) is a rare mesenchyma skin tumor. It is characterized by a slow growth with a high rate of recurrence but limited potential for metastasis. We report the result of a retrospective study of 18 cases of DFSP collected over 16 years in the dermatology department of La Rabta Hospital. The mean age was 32 years with a sex ratio of 1.57. The site of predilection was the trunk (77.5%). Histological features were typical in all cases with positivity for CD34 in 14 cases. Treatment was surgical in 13 cases. DFSP is a tumor of intermediate malignancy, which can have aggressive course. This tumor is best treated with surgery in which large excision are necessary to reduce the risk of recurrence.
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