We would like to share two cases with combination of harlequin ichthyosis (HI) and JIA. Harlequin Ichthyosis has prevalence of 1/1000 000 and incidence of 5 births/ year in the UK with an overall survival rate of 56%. The molecular pathogenesis includes hyperkeratinosis of skin resulting in significant contractures with fixed flexion deformities and underdevelopment with short stature besides other clinical features. It is linked to ABCA12 mutation but heterozygous mutation, Oral retinoids and

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... dvanced medical management had led to increased survival. Thus, unfolding varied rheumatological manifestations which adolescent rheumatologists are more likely to encounter in future. Methods: In case 1, a 30 year old woman with diagnosis of HI since birth and rickets as a child was referred to musculoskeletal services in 2002 with ongoing painful elbows, shoulders, fingers and locking of wrists for few years. On examination, symptoms suggestive of impingement with marked restriction of joint movements with fixed flexion deformities of fingers were noted. Radiographic features included significant destructive changes around wrist with subluxation of proximal row of carpal bones. An MRI scan of shoulder revealed rotator cuff tendinopathy and small joint effusion but no synovitis. She remains seronegative and is currently being managed by intensive physiotherapy, splints and intra-articular injections. To the best of our knowledge, she is the longest known survivor of this condition in the world and surely in the UK. In case 2, a 22 year old man with HI was seen in our adolescent rheumatological services since age of 16. He was diagnosed with JIA in Addenbrooke's, Cambridge and his inflammatory arthritis was managed in early years with MTX. He was soon commenced on etanercept at 25 mg/ week which revolutionized his inflammatory arthritis and he no longer needed a wheelchair to mobilize. He was subsequently tried on adalimumab, MTX and is currently on LEF. He has reported worsening of his skin condition with cracks and increased skin infections with all previous DMARDs and biologics and his management remains a clinical challenge. Results: To the best of our knowledge three cases of inflammatory arthritis with HI have been reported in literature, our male patient being one of them (Clements et al). Little has been researched about biochemical changes in inflammatory cytokines in patients with HI, certainly a mouse model with overexpression of IL-37 hypothesizing inflammation as contributor to HI. Conclusion: Even little is known about management of inflammatory arthritis in patients with HI and effects of conventional DMARDs and biologics on dermatological manifestations. With increasing life expectancy in HI we can expect to see an increase in metabolic bone disorders, increased degenerative joint disease and indeed more of inflammatory arthritis which will pose a diagnostic and management challenge. Disclosure statement: The authors have declared no conflicts of interest.