Lichen planus pemphigoides (LPP) is a rare autoimmune blistering disease. It appears to be combination of lichen planus and bullous pemphigoid. We describe four new cases of LPP and discuss the epidemiological, clinical, pathological, and therapeutic features of this singular association through a review of the 74 published cases within the English literature. We report four cases of LPP (three women aged respectively 47, 51, and 53 years old, and a 53-year-old man). All patients presented with bullae on lichenoid and normal skin, predominately on the extremities. The diagnosis was confirmed by immunohistological findings. Our patients were treated with oral corticosteroids with a good response. Our review of the literature of 78 cases of LPP (65 adults and 13 children) showed that it involved adults (mean age: 54 years), with a slight female preponderance. A mean lag time between LP and the development of LPP was 8.3 months. LPP is characterized by developing blisters on lichenoid lesions and on uninvolved skin with more acral distribution of bullous lesions. Involvement of palms and soles was more frequent in children. The diagnosis is based on pathological and immunological confrontation. LPP is usually idiopathic, but some cases were reported in association with various drugs. There have also been reports of association with internal malignancy. Most cases of LPP are successfully treated with systemic corticosteroids. In most cases, the prognosis was good.

The P Veg seems to be more frequent in Tunisia with high rate of mortality.

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662Cerebellar liponeurocytoma (LPN) is a rare central nervous system tumour recently recognized as a distinct clinicopathological entity. It was included in the previous 2000 edition of the World Health Organization (WHO) classification as a grade I neoplasm under the heading of glioneuronal tumours 1 . With increasing recognition of this entity and longer clinical follow-up periods in its investigation, it has become clear that this tumour has a rate of recurrence that is higher than previously thought and not compatible with a grade I designation. In light of the substantial rate of tumour recurrences, the current 2007 WHO classification assigns the cerebellar liponeurocytoma to WHO grade II tumour 2 . In this paper, we report a new case of a recurrent cerebellar LPN with supratentorial extension despite persistently benign histological features. To the best of our knowledge, recurrent cerebellar LPN with supratentorial extension has never been reported before, as the nine cases of recurrent cerebellar LPN published to date were confined to the posterior fossa. CLINICAL HISTORYA 42-year-old previously healthy man, presented with a fivemonth history of headaches, vertigo, visual disturbance and progressive unsteadiness of gait. On admission, neurological examination showed signs of cerebellar dysfunction, including ataxia, wide-based gait, right dysmetria on finger-to-nose testing and a positive Romberg sign. The fundus examination revealed unilateral papilloedema. Computed tomogram scan ( Figure 1) demonstrated a hypodense lesion within the vermis and right cerebellar hemisphere compressing the 4th ventricle and enhancing after contrast injection. Magnetic resonance imaging (MRI) scan showed a heterogeneous mass measuring 5 × 4 cm, characterized by discrete hyperintensity on T1-weighted images and high signal intensity on T2-weighted images. Several central parts of the lesion were very hyperintense on both T1 and T2-weighted sequences suggesting fat content. An intra-operative frozen section was reported as being consistent with a medulloblastoma. A gross total resection of the tumour was achieved. Histological examination of the surgical specimen revealed cerebellar tissue infiltrated by a highly cellular tumour formed by sheets of isomorphic small round cells (Figure 2). A significant proportion of the tumour demonstrated lipomatous differentiation characterized by large areas of cells containing a single cytoplasmic vacuole displacing the nucleus to the periphery mimicking mature fat cells (Figure 2A). There were only occasional mitotic figures and a low rate of proliferation Figure 2B) and neuron-specific enolase. Glial fibrillary acidic protein immunopositive tumour cells confirmed the presence of an additional glial component. All of these features were considered to be consistent with the diagnosis of cerebellar liponeurocytoma. A post-operative MRI performed three days after the surgery, demonstrated no residual tumour. No adjuvant radiation therapy was administered post-operatively. At the last rout...

Basal cell carcinoma (BCC) is the prototypical basaloid tumor of the skin. It may show various patterns simulating other cutaneous tumors due to its pleomorphism. It may have an unusal pattern of differentiation such as squamous, sebaceous, apocrine, eccrine, pilar, and endocrine differentiation. In order to establish the relative frequency of neuroendocrine differentiation in BCC, we performed a retrospective study of 33 consecutive BCCs using conventional immunohistochemistry with two neuroendocrine antibodies: Chromogranine A and synaptophysine. The age of the patients ranged from 17-83 years with mean of 65 years. The male to female ratio was 16:17. In immunohistochimestry, Chromogranine A was seen in 72.2% (24/33) while Synaptophysine was positive in 9.09% (3/33). Their expression was cytoplasmic and membranous and was seen in the periphery of these tumors in the overlying cells. Positive staining of chromogranine A was high (75-100% of tumors cells) in 9%, intermediate (25-75% of tumors cells) in 33% of cases and relatively low (<25%) in 30.3% of cases.

Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease however is a rare. In this report, we present an unusual case of neurosarcoidosis with intra cranial mass mimicking radiologically a glioma. Pathological examination revealed intraparenchymatous necrotising granulomatous lesions. After clinicopathological correlation, the diagnosis of a necrotizing cerebral granulomatosis (neurosarcoidosis) with atypical systemic involvement was made. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease.

Introduction: Central nervous system tumours represent 20% of all childhood cancers, and are the second most common group of neoplasms after leukaemias. Objective: To describe epidemiological characteristics of central nervous system tumours in a paediatric Tunisian population. Patients and Methods: A retrospective study of 492 childhood central nervous system tumours operated between 1990 and 2004 was undertaken. We investigated the age-related location, gender distribution and the histology of all tumours, and adopted the latest WHO classification (2007) in grouping all the tumours. Results: There were 488 primary and 4 secondary tumours; 426 (86.6%) were intracranial and 66 (13.4%) were intraspinal. Of the 426 intracranial tumours, 214 (50.24%) were supratentorial and 212 (49.76%) were infratentorial. The median age at diagnosis was 8 years, with a male:female ratio of 1.14:1. Low-grade tumours (WHO I/II) constituted 67.3% of all lesions and the rest (32.7%) were high-grade tumours (WHO III/IV). The most common tumour found in our series was astrocytoma (38%), followed by medulloblastoma (16.2%), then ependymoma (6.9%), cystic tumours (6.3%) and craniopharyngioma (5.3%). The overall 5-year survival rate was 45% with a mean follow-up period of 36 months. Conclusion: In our patient population, the incidence and distribution of central nervous system tumours were similar to those reported in literature. Overall survival rates varied according to tumour location and histopathology.