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Japanese Journal of Clinical Immunology
Nakajo-Nishimura syndrome (NNS) (MIM256040, ORPHA2615) is a distinct inherited in‰ammatory and wasting disease, which usually begins in early infancy with a pernio-like rash. The patients develop periodic high fever and nodular erythema-like eruptions, and gradually progress lipomuscular atrophy in the upper body, mainly the face and the upper extremities, to show the characteristic long clubbedˆngers with joint contractures. So far about 30 cases have been reported from Kansai, especiallydoi:10.2177/jsci.34.388 pmid:22041427 fatcat:tywkmxhgqrg5zdipeesrgsjzim