Dyskeratosis congenita: a case report and review of literature

Dyskeratosis congenita (DC) is classically characterised by a mucocutaneous triad of reticulated poikiloderma, nail dystrophy and mucosal leukoplakia together with bone marrow failure and increased risk of malignancy1- 4. Due to its rarity and clinical heterogeneity it is not easily recognised and patients are often treated for other entities. We report a case of dyskeratosis congenita who presented to us with the classical triad in his late twenties after years of being treated as lichen planus

Successful treatment of paediatric generalized pustular psoriasis with secukinumab: a case series

Generalized Pustular Psoriasis (GPP) is a rare, severe, life-threatening form of psoriasis and accounts for up to 13.1% of all childhood psoriasis. Common first-line systemic treatment for pediatric patients with GPP include oral acitretin, cyclosporin and methotrexate which have varying efficacy and side effects but multiple interventions are often needed to induce remission and maintain long term control. Recently, the anti IL 17 A monoclonal antibody secukinumab have been shown to be effective in adult patients with GPP; however, there is lack of evidence of its usage in the pediatric population. We describe a case series of 4 pediatric patients with GPP who were treated with off-label use of secukinumab. All four patients had marked clearance and reduction in Generalized Pustular Psoriasis Area & Severity Score (GPPASI) within first 48 h of first injection with subsequent almost complete to complete clearance of skin lesions by 1 month follow up. In conclusion, secukinumab was found to be successful in inducing remission, with rapid clearance and maintaining remission, with or without combination with other systemic agents for pediatric GPP