Inherited condition affecting skin, eyes and digits.
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