[Sebaceous linear nevus syndrome with hemimegalencephaly. Report of a case]. / Sindrome del nevo lineare sebaceo con emimegalencefalia. Su di un caso.

The case of a 7-year-old girl, born from a monochorionic biamniotic pregnancy (with healthy male twin) is presented. The patient showed a congenital brown-yellowish, raised, rough and oval-shaped nevic neoformation of the right temporo-zygomatic region. At 28 days of age she had a right hemiclonic status epilepticus, and from the 8th month of life she presented right sided partial motor seizures. Moreover from the beginning, her development milestones were delayed. When she was 5 years old an electroencephalogram displayed a sharp asymmetrical background activity, a continuous paroxysmal activity on the right hemisphere and independent focal irritative anomalies on the left posterior region. Her computed tomographic scan demonstrated megalencephaly on the right hemisphere. A biopsy specimen of her skin lesion showed the histological characteristics of a nevus sebaceous of Jadassohn. Taken together the clinical, neuroradiological and dermatological data led to the diagnosis of linear nevus sebaceous syndrome.

Safety of valproate in porphyria cutanea tarda.

Treatment of epileptic seizures in patients with hepatic porphyrias is a challenging problem due to enzymatic induction activity of phenobarbital (PB), phenytoin (PHT), carbamazepine (CBZ), and clonazepam (CZP). We present the case of a patient with partial seizures treated with PHT and showing clinical signs and biochemical abnormalities of porphyria cutanea tarda (PCT). We withdrew PHT and treated the patient with sodium valproate (VPA). We followed the patient for 6 months during VPA therapy. During this period, clinical signs of PCT disappeared and biochemical values normalized. Our study shows that VPA is a safe treatment in epileptic patients with PCT.

Cytoskeleton and extracellular matrix of cutaneous vessels in inflammatory processes: immunomorphological study.

In pathological conditions, vascular modifications occur in various stages involving both vessel structure and adjacent extracellular matrix. The relationships between vascular cells and surrounding microenvironmental stroma are mediated by cytoskeleton. Our investigation showed a high number of vimentin- and actin-positive cells in the vascular cutaneous bed, mainly related to reactive vascularization phenomena, whereas vessel cells with a desmin-positive reaction were barely detectable. Furthermore, in newly formed vessels ultrastructure showed that basement membrane synthesis strictly depends on close contact between the endothelium and extracellular matrix. Our data give structural evidence of the close morphofunctional interactions existing between vascular cells and extracellular matrix.