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Inherited condition affecting skin, eyes and digits.

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CASS.

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Described in only one family with 5 affected siblings on Rodrigues Island in the Indian ocean (approximately 1600 km east of Madagascar). Autosomal recessive.

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Sclerodactyly, hyperkeratosis, digital contractures, and pseudo-Ainhum syndrome (digital constriction bands with autoamputation) associated with cataract and total alopecia.

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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.

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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]

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