Primary hypertrophic osteoarthropathy.
We describe seven patients with primary HOA and review 125 cases reported in the English, French, and German literature. The salient clinical features of primary HOA are: a bimodal distribution of disease onset with one peak during the first year of life and the other at age 15, a male predominance (nine to one), uncommon benign joint effusion, and a variety of skin abnormalities resulting from cutaneous hypertrophy or glandular dysfunction. We concluded that HOA is not a synovial disease. It is suggested that synovial effusions, when present, are perhaps a sympathetic reaction to the neighboring periostitis. Proposed diagnostic criteria for HOA, including digital clubbing and radiographic periostitis, appear 86% sensitive. The clinical features, age of onset, and sex distribution suggest that a genetically controlled growth promoting factor, different from growth hormone, plays a role in the pathogenesis of this syndrome.
Martínez-Lavín
M
M
Instituto Nacional de Cardiología Ignacio Chávez, Mexico, D.F.
Pineda
C
C
Valdez
T
T
Cajigas
J C
JC
Weisman
M
M
Gerber
N
N
Steigler
D
D
D000293:Adolescent; D000328:Adult; D002648:Child; D006801:Humans; D008297:Male; D010004:Osteoarthropathy, Primary Hypertrophic / Q000175:diagnosis*; D009626:Terminology as Topic