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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 osteophytic mass.

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 28:518, 1969.  [PubMed: 5346342]
Crosby ET, Grahovac S: Diffuse idiopathic skeletal hyperostosis: An unusual cause of difficult intubation. Can J Anaesth 40:54, 1993.  [PubMed: 8425244]
Kiss C, Szilagyi M, Paksy A, et al: Risk factors for diffuse idiopathic skeletal hyperostosis: A case-control study. Rheumatology (Oxford) 41:27, 2002.  [PubMed: 11792876]

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