“…In adults, the median age of symptom onset is 17 years, with an estimated prevalence of 0.9 per 100,000 people and an average incidence of 0.23 per 100,000 people according to another Japanese survey [8,9]. CIPO is usually idiopathic in children, while in adults, approximately 60% of the cases are secondary to an underlying condition such as scleroderma, systemic lupus erythematosus, dermatopolymyositis, malignancy and amyloidosis [2,3]. Familial forms of CIPO, which may be inherited as autosomal dominant, autosomal recessive or X-linked traits, have also been described [2].…”