Neurocutaneous melanosis (NCM) is a very uncommon, nonfamilial "neurocutaneous syndrome." It is characterized by the presence of "multiple or large congenital nevi" with central nervous system (CNS) melanocytic deposits. [1] Rokitansky published the first report of NCM in 1861. In his report, he described a mentally disabled girl who was born with a "large congenital melanocytic nevus" and eventually died due to hydrocephalus complications. At autopsy, he found that she had brain melanocytic accumulations. [2] It was named NCM in 1948 by Van Bogaert. The accurate incident of NCM is unidentified. It is very rare, with only about 100-200 cases reported. Both sexes are affected equally, and there is no racial or ethnic predominance identified.

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BackgroundThe treatment of children with rheumatic and musculoskeletal diseases (RMD) have changed significantly with biologic treatment.ObjectivesTo look at reasons for switching from one biologic to another.MethodsPatients with confirmed RMD identified in our centre using The International Classification of Diseases, 9th Revision, Clinical Modification (ICD-9-CM), from 1st January 2011 to 31st December 2015. Retrospective review of electronic records and paper case notes of patients presented to the centre reviewed using pre-specified Clinical Research Form (CRF).ResultsWe identified 181 paediatric patients on continuous follow up. The distribution as follows; juvenile Idiopathic Arthritis (JIA) 78, Systemic Lupus (JSLE) 19, Juvenile Scleroderma (Scl) 5, vasculitis 7, Juvenile Dermatomyositis (JDM) 6, Familial Mediterranean Fever (FMF) 9, Macrophage Activation Syndrome (MAS) 3 and Uveitis only in 2. Other diagnoses of non-inflammatory nature 52 including juvenile osteoporosis, mechanical pain syndromes and vitamin D deficiency.The total number of patients on biological therapy is 35 patients including 33 JIA and 2 JSLE. The number of patients still on the same biologic agent is 25.The following are the biologics with numbers of patients still on the same therapy (25): Etanercept 7 (JIA), Infliximab 3 (JIA), Adalimumab 1 (Uveitis), Abatacept 1 (JIA), Tocilizumab 7 (JIA), Anakina 4 (JIA 3, FMF 1), Rituximab 2 (JSLE).The number of patients who switched their treatment to a second or third agent is 10. The reason for switching biological treatment was lack of efficacy in 7 out of the 10 patients and side effects in the other 3 patients (table 1). Average follow up on current treatment of 36.6 months (range 0.5 to 81 months).Table 1Case NumberDiagnosis1st biologic2nd Biologic3rd BiologicReason for switching1Oligo JIA + UveitisAdalimumabInfliximabPersistent eye inflammation2Extended JIAEtanerceptTocilizumabPersistent joint inflammation3Polyarticular JIAAdalimumabAbataceptPersistent joint inflammation4Polyarticular JIAAdalimumabTocilizumabAbataceptPersistent joint inflammation in 2nd and 3rd5Polyarticular JIAEtanerceptAdalimumabUveitis with etanercept6Extended Oligo JIAAbataceptTocilizumabPersistent inflammation7Systemic JIATocilizumabCanakinumabAnakinraPersistent inflammation with 1st biologic MAS twice on Canakinumab8Enthesitis Related JIAEtanerceptAdalimumabUveitis on Etanercept9Poly JIAAdalimumabTocilizumabPersistent joint inflammation10Systemic JIAEtanerceptTocilizumabPersistent inflammationConclusionsThe percentage of patients who switched biological treatment is 28.6% although numbers are small. Reasons for switching biologic treatment in our experience in children with RMD is:-lack of efficacy with persistent inflammation in 7 out of 10.-Side effects of treatment in 3 out of the 10 (Uveitis while on etanercept (2) and one developed MAS twice on canakinumab.AcknowledgementTo all the children and their families for allowing us to use their data.Disclosure of InterestNone declared

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