[Liposarcoma of the thumb].
Liposarcoma is the most common malignant deep soft-tissue tumor in adults. however, primitive cutaneous liposarcoma is very rare. We report the observation of a patient who presents a liposarcoma of the thumb.
Humans↗
Biomedical subjects
L Benzekri
Publications and source records attributed to L Benzekri.
[Porphyria cutanea tarda in a hemodialysed patient with hepatitis C virus infection: efficacy treatment by small repeated phlebotomies].
INTRODUCTION: Porphyria cutanea tarda (PCT) is a disorder of heme biosynthesis resulting from deficiency in the enzyme uroporphyrinogen decarboxylase. In the sporadic form of PCT, there are many agents that trigger the clinical manifestations. EXEGESIS: We report a case of PCT in an hemodialysed patient with hepatitis C virus infection (HVC). He was treated with small repeated phlebotomies of 50 ml every week with photoprotection, eviction of traumatismes and inducing drugs. A clinical remission was induced after five months of treatment. CONCLUSION: A proper diagnosis of PCT in non uremic hemodialysed patients requires fractionation of serum and fecal porphyrin changes. Management of this patients is difficult. Small repeated phlebotomies (50-100 ml) could be an interesting therapy.
Aged↗
[Perifolliculitis capitis abscedens and suffodiens].
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Abscess↗
[Maffucci syndrome associated with epidermoid carcinoma of the nasopharynx].
BACKGROUND: Maffucci's syndrome is a dysembryoplasia of the mesoderm, explaining the dual involvement of cartilage and vascular tissue. The risk of malignant degeneration or associated tumors is high in this uncommon disease. We report a case of Maffucci's syndrome associated with squamous cell carcinoma of the cavum. CASE REPORT: A 37-year-old man consulted for multiple angiomas and chondromas which had developed since childhood. The diagnosis of Maffucci's syndrome was obvious. Radiological exploration of the limbs confirmed chondromatosis and biopsy of the cavum performed because of the occurrence of epistaxis, revealed squamous cell carcinoma. DISCUSSION: Maffucci's angiochondromatosis is a rare, non-hereditary but sometimes congenital disease. Angiomas predominate on the hands and feet. Sarcomatous degeneration is the main complication of these chondromas. Angiomas rarely become malignant, but when they do are generally more aggressive than chondromas. The frequency of neoplasia in Maffucci's syndrome would suggest that there is a supplementary oncogenic factor. Several types of malignancy associated with Maffucci's syndrome have been reported, but to our knowledge there has been no publication concerning an association between Maffucci's syndrome and squamous cell carcinoma of the cavum.
Adult↗
[Atypical presentations of leprosy: apropos of 2 cases].
This report describes two atypical cases of leprosy. A 48 year old male patient presented laryngeal dyspnea with adhesions of the oropharynx of which the biopsies were inconclusive. The patient was cachectic with hyperesthesia of the extremities and two subcutaneous nodules. The biopsy of one nodule evoked thesaurismosis or dyslipoidosis while the bacilloscopy was positive in nasal smears. A 14 year old female patient suffered from bullae which appeared spontaneously on erythematous skin on the legs and upper arms. Upon examination those areas were found to be hypoaesthetic, as was a very large hamartoma on the left half of the body. A biopsy of healthy skin evoked the diagnosis of leprosy. The patient then developed BT leprosy and episodes of hysteria. The first observation led to several diagnoses: while laryngeal dyspnea is unusual in LL and while cutaneous histology of regressive LL contrasted with the abundance of the bacilloscopy. The diagnosis of the second case is that of indeterminate leprosy with premonitory neurological signs associated with pathomania and evolution to a multibacillary form.
Adolescent↗
[Closed spinal dysraphism. Apropos of 3 cases].
CASE REPORT: Closed spinal midline developmental defect was observed in three children, two girls aged 7 and 10 years and one boy aged 9 years. There were no similar cases in the families. The first signs appeared at approximately 6 months, 9 years and at birth. In the first two cases, developmental defects of the toes were followed a few months or a few years later by chronic ulcerations of the plantar surface of the foot. On physical examination there was a greater toe, amputation of the toes, perforating plantar ulceration, prolonged cauda equin and neurological disorders. Radiology revealed defective closure of the dorsal and/or lumbar vertebrae and lysis of the third phalanxes and magnetic resonance imaging showed diastematomyelia. The third child was born with a pilous nevus and a prolonged cauda equin. A soft subcutaneous tumor developed 3 years later over the lumbar area, increasing in size becoming quite voluminous and painful. In this case, there was no amputation of the toes or perforating plantar ulceration or neurological disorders. Radiography showed hemisacralization of L5 and spina bifida of S1. Magnetic resonance imaging showed a normal spinal cord with a tumor independent of the neural canal. Biopsy exeresis led to the diagnosis of neurofibroma. DISCUSSION: Closed spinal midline development defect occurs more readily in girls than in boys. Multiple and complex abnormalities are observed: diastematomyelia, diplomyelia, dermal fistulae, neuroenteral cysts, dysembryoplasic tumors, abnormal position of the spinal cord, and usually vertebral malformations. There are three types of clinical signs involving skin, bone and neurological manifestations. Radiological explorations should be completed with myelography and a computed tomography as well as magnetic resonance imaging to determine the extent of the malformations and possible surgical indications.
Child↗
[Leg ulcer in childhood and adolescent: 31 cases].
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Adolescent↗
[Werner syndrome. Apropos of a case].
A 26 year-old woman, whose parents were consanguineously married, was admitted to our center because of bilateral juvenile cataract. The patient exhibited short stature, sclerodermalike appearance of the skin with a typical bird-like facies, thinning and graying of hair, high pitched voice and hypogonadism. Werner's syndrome, was diagnosed. History, pathogeny, clinical features, diagnosis and cataract surgery are discussed.
Adult↗