Inherited epidermolysis bullosa dystrophica and squamous cell carcinoma- A case report.

Epidermolysis bullosa dystrophica (EBD) is an inherited disease of the structural proteins in the upper dermis, characterized by blister formation at the site of trauma followed by scarring. Skin fragility and blistering are the hallmarks of this disease. Cutaneous squamous cell carcinoma (cSCC) is a dreadful complication in the epidermolysis bullosa (EB) patients and common cause of death. The recent advances in distinct tumor microenvironment explain the aggressive nature of SCC in recessive Recessive Dystrophic Epidermolysis Bullosa (RDEB) patients and the use of collagen VII re-expression as a possible therapeutic measure. Regular follow-up is a must in preventing complications.

Assessment of behavioural problems in preschool and school going children with epilepsy.

Introduction: Children with epilepsy are at greater risk of developing psychiatric and behavioural disorders such as attention deficit/hyperactivity disorder (ADHD), conduct disorder, autism spectrum disorder (ASD), as well as affective and aggressive disorders than normal children which may affect the well- being and quality of life of the child. Aim and Objectives: This study aims at identifying behavioural problems in children with epilepsy enabling early diagnosis and intervention. The objectives were to assess the presence and type of behavioural problems in children with epilepsy. Methods: A prospective cross-sectional study was conducted on children who were diagnosed as epilepsy in two age groups of 1.5-5 years and 6-18 years recruited by non-probability convenience sampling. Data regarding seizure semiology, clinical features and treatment were obtained. Children underwent IQ assessment, electroencephalogram and brain neuroimaging. Child Behaviour Check List (CBCL) was administered to parents or primary caregivers after obtaining informed consent. Results were analyzed for presence of behavioural problems using SPSS-23. Results: In the study, out of 50 study subjects, 72% were between 6-18 years. 60% children had generalised seizures, 58% children had epilepsy for <2 years and abnormal EEG was present in 80% children. 6% children had behavioural problems and 4% had borderline presentations. Co-relation of behavioural problems with age was statistically significant with p value 0.027. Behavioural problems identified were aggressiveness and anxiety. Conclusion: Childhood epilepsy is associated with behavioural problems along with other co-morbidities warranting a search during follow-up visits. Take-home message: Early identification and treatment of behavioural problems in children with epilepsy by periodic assessment during follow up visits, careful selection of combination of drugs and appropriate dose can improve the overall outcome in children taking antiepileptic drugs (AEDs) for epilepsy.