[Brachioradial pruritus revealing an ependymoma]. / Prurit brachioradial révélant un épendymome.

BACKGROUND: Brachioradial pruritus is a rare form of pruritus localised to one or more brachioradial dermatomes, initially classified as a photodermatosis but which in fact is generally brought on by nervous compression. We report a case of a brachioradial pruritus revealing an intramedullary tumour. PATIENTS AND METHODS: A 53-year-old man had presented pruritus for seven years under the left clavicle, then on the left forearm followed by the right forearm. Finally cervicodynia appeared associated with dysaesthesia of the two upper limbs, fulgurating pains and paresis of the left cubital region. The examination revealed suspended bilateral hypoaesthesia (C4, C5, C6, C7), proprioceptive disorders of the left upper limb, mild motor deficit in the left C8 area and tetrapyramidal syndrome. Cervical radiography did not show cervical osteoarthritis. The MRI revealed a bulky cervical intramedullary tumour extending from C2 to C6. After ruling out cavernoma by medullary angiography, surgery was performed and histopathological analysis of the complete lesion revealed a benign ependymoma. Four months later, this patient complained about residual pains requiring treatment with gabapentin and class 2 analgesics. DISCUSSION: The case presented underlines the possibility of a brachioradial pruritus revealing an intramedullary tumour. Ependymomas are usually seen in children and are frequently evoked in the presence of dysaesthesia. We report the third case of brachioradial pruritus revealing a medullary tumour. The two other cases involved syringomyelia revealed by pruritus in C5 and ependymoma revealed by pruritus in C5-C6. The patient with ependymoma had refused surgical treatment. CONCLUSION: Atypical brachioradial pruritus complicated by neuropathic pains and disorders should prompt screening for a medullary tumour.

Diffuse EGFR staining is associated with reduced overall survival in locally advanced oesophageal squamous cell cancer.

Squamous cell carcinoma of the oesophagus (SCCO) is still a pathology of bad prognosis. Specific therapies are now developed against epidermal growth factor receptor (EGFR), human epidermal growth factor receptor 2, c-kit receptor (CD117), vascular endothelial growth factor (VEGF) and p53 protein. This study was aimed at assessing their expression in a large series of SCCO, as well as their potential therapeutic interest in this pathology. Immunohistochemical expression of these factors was assessed retrospectively in 107 cases of SCCO with primary surgery, as well as their relationships to recurrence, metastasis and overall survival on a long-term follow-up. Human epidermal growth factor receptor 2 and CD117 were expressed in less than 3% of the cases. Epidermal growth factor receptor and p53 were overexpressed in 68.2 and 66.4% of the cases, and VEGF in 38.3%. Epidermal growth factor receptor overexpression was significantly related to vascular invasion (P=0.023). Its diffuse positivity was significantly related in multivariate analysis to higher local recurrence (P=0.006) and lower overall survival (P=0.003), in a subgroup of patients of poor outcome who had received postoperative adjuvant treatment. These results highlight the great potential prognostic and therapeutic interest of evaluating EGFR diffuse positivity in locally advanced SCCO.

FDG PET in epithelioid hemangioendothelioma.

Epithelioid hemangioendothelioma (EH) is an uncommon tumor of endothelial origin. It can develop in any tissue and can be multicentric or metastatic. The usual course is a slow progression. Imaging techniques are generally useful in determining the extent of the disease. A case of EH involving bone marrow and mediastinum is described. We discuss the use of FDG PET scanning in EH, showing its use in detecting bone marrow involvement and determining the extent of the disease.

[Trumpet nail deformity during the course of Dowling-Meara type epidermolysis bullosa simplex. A report of two cases]. / Ongles en cornet au cours de l'épidermolyse bulleuse de Dowling Meara: 2 cas.

INTRODUCTION: We report two patients with Dowling-Meara type epidermolysis bullosa simplex: who had trumpet nail deformity. CASE REPORTS: Two 75 and 72 year-old sisters, had flare-ups of epidermolysis bullosa simplex since childhood. The aspect of the lesions and electron microscopy were in favour of the diagnosis of the Dowling-Meara variant of epidermolysis bullosa simplex. Both women had several fingernails with pincer and trumpet nail deformities. There were no such signs among the other siblings, who had never had epidermolysis. DISCUSSION: Nail involvement is not rare during the course of superficial epidermolysis bullosa. The trumpet lesions appear to be relatively specific to the present two cases of epidermolysis bullosa simplex. The electron microscopic findings of these two cases were also noteworthy, with whisk-like but not round clumping of the perinuclear tonofilaments.