Main clinical features
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characterized by spontaneous episodes of subperiosteal new bone formation along 1 or more bones commencing within the first 5 months of life, acute manifestations inflammatory in nature, with fever and hot, tender swelling of involved bones (e.g., mandible, ribs)
hyperirritability, acute inflammation of soft tissues, and massive subperiosteal new bone formation; typically appears in early infancy and is considered a benign self-limiting disease.
associated to joint hyperlaxity, hyperextensible skin, and inguinal hernias resembling symptoms of a mild form of Ehlers-Danlos syndrome type III
in any cases, short stature and persistent bony deformities |