Genetic disorder with ankylosis of the spine and
hyperkeratosis of palms and soles.
Ankylosing Vertebral Hyperostosis with Tylosis.
One Greek Cypriot family has
been described. Because six members of the sibship had tylosis
(hyperkeratosis punctata plantaris and palmaris) alone, two independent
genetic traits may have been present.
All the affected individuals had ankylosing
vertebral hyperostosis, including ossification of paraspinal ligaments and
formation of large osteophytes. Most were asymptomatic; a few complained
about low-grade back pain. Tylosis was present in all patients. One member
had mild psoriasis. The osseous manifestations are basically identical to
those found in diffuse idiopathic skeletal hyperostosis (DISH), and some
researchers use Beardwell and Forestier syndrome synonymously. However,
although onset of DISH (ankylosing hyperostosis, asymmetrical skeletal hyperostosis or senile ankylosing hyperostosis) before 50 years of
age is exceedingly rare, affected individuals here were between 18 and 50 years
old at the time of the report. DISH often is asymptomatic, but many of the
different symptoms and complications reported are dependent on the location
of the osteophytes. The symptoms may range from pain and stiffness to
stridor and difficulties swallowing. Furthermore, a higher incidence of
diabetes mellitus and other metabolic disorders has been reported.
Most of the individuals described by
Beardwell were asymptomatic, and implications for anesthesia have not been
described. However, anesthetic complications in DISH patients have been
reported, for example, difficult tracheal intubation from deviation and
stenosis of the trachea, decreased neck mobility, vocal cord paresis, and
one case requiring emergency tracheostomy for severe airway obstruction caused by an
Forestier Disease (Diffuse Idiopathic Skeletal Hyperostosis, DISH): The
bony lesions are phenotypically similar. The incidence of DISH is higher,
and it has been described in Caucasians and Africans.
Keratosis Palmaris et Plantaris: Other forms of palmoplantor hyperkeratosis
are summarized under this title.
Beardwell A: Familial ankylosing vertebral hyperostosis with tylosis.
Ann Rheum Dis
Crosby ET, Grahovac S: Diffuse idiopathic skeletal hyperostosis: An unusual
cause of difficult intubation. Can J Anaesth
Kiss C, Szilagyi M, Paksy A, et al: Risk factors for diffuse idiopathic
skeletal hyperostosis: A case-control study. Rheumatology (Oxford)