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Hypertrophic Osteoarthropathy (HOA)

HOA (also known as Pierre-Marie syndrome, Bamberger syndrome, osteoarthropatia hypertrophica, Mankowsky syndrome and Hagner syndrome) is a syndrome that includes clubbing of fingers (Hippocratic fingers) and toes, periostitis of tubular bones, and arthritis with a noninflammatory synovial fluid.

Primary HOA (pachydermoperiostosis) is autosomal dominant, appears during childhood mostly in males and is characterized by clubbing, skin hypertrophy (pachyderma), coarse facial features, seborrhea, and hyperhidrosis.

Secondary HOA can be divided into generalized or localized form.

Generalized form: often associated with intrathoracic malignant neoplasms (nonsmall cell lung cancer, mesothelioma, and others) in 90% of cases. It also occurs in patients with other malignancies (hepatic, gastrointestinal, POEMS), chronic infections (lung, subacute bacterial endocarditis, HIV, and others), cystic fibrosis, congenital cyanotic heart disease, inflammatory bowel disease, cirrhosis, Graves’ disease, or on certain drugs (voriconazole).

Localized form: associated with hemiplegia, aneurysms, infective arteritis, and patent ductus arteriosus.

The clinical course of secondary HOA is determined by the underlying primary disease. Platelet/endothelial cell activation with release of vascular endothelial growth factor and platelet-derived growth factor appears to play a key role in the etiology of HOA.


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