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Adult‐Onset Idiopathic Progressive Acro‐Osteolysis With Proximal Symphalangism
Author(s) -
Tanikawa Takahisa,
Okada Yosuke,
Azuma Taeko,
Fukushima Ayumi,
Kawahara Chie,
Tanaka Yoshiya
Publication year - 2004
Publication title -
journal of bone and mineral research
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.882
H-Index - 241
eISSN - 1523-4681
pISSN - 0884-0431
DOI - 10.1359/jbmr.0301210
Subject(s) - medicine , osteolysis , surgery , anatomy
We experienced a 57‐year‐old female with adult‐onset non‐congenital idiopathic acro‐osteolysis combined with proximal symphalangism. At the age of 36, she developed severe pain and swelling of the toe base of both feet and underwent Clayton surgery. However, the size of her toes diminished progressively over the 5‐year period after surgery. At the age of 41, she suffered pain and swelling of the proximal interphalangeal (PIP) joints of fingers of both hands. These PIP joints became rigid and inflexible. Subsequently, she noticed shortening of the little finger of both hands, followed later by shortening of the index, middle, and ring fingers. At the age of 57, the thumbs began to shorten. Laboratory and endocrinological examinations were not abnormal. Finally, we diagnosed her with acro‐osteolysis combined with proximal symphalangism by radiological examination. In this case, previously unreported mutations of the Noggin gene were identified. This is the first case report of adult‐onset, non‐congenital idiopathic acro‐osteolysis combined with proximal symphalangism.