++
Inherited condition affecting skin, eyes and digits.
++
++
Described in
only one family with 5 affected siblings on Rodrigues Island in the Indian ocean
(approximately 1600 km east of Madagascar). Autosomal recessive.
++
Sclerodactyly, hyperkeratosis, digital
contractures, and pseudo-Ainhum syndrome (digital constriction bands with
autoamputation) associated with cataract and total alopecia.
++
Anesthesia in this condition has not
been described. Theoretically, the digital features may limit the patient's
ability to activate certain devices, such as a pump for patient controlled analgesia.
++
Ainhum: A narrow strip of hardened skin with constricting ring
formation on the little toe at the level of digitoplantar fold progressively
leading to spontaneous amputation.
Wallis C, Ip FSL, Beighton P: Cataracts, alopecia, and sclerodactyly:
A previously apparently undescribed ectodermal dysplasia syndrome on the
island of Rodrigues.
Am J Med Genet 32:500, 1989.
[PubMed: 2773993]