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Salivary Pleomorphic Adenoma in Familial Adenomatous Polyposis

sweat in response to these tests-evidence suggesting a post-synaptic lack, or malfunction, in the cholinergic receptors. Once the association of sensory loss and anhidrosis is recognized the diagnosis can be in little doubt. The anhidrosis of ectodermal dysplasia is excluded by the clinical features of nail and hair abnormalities as well as the absence of sweat glands on skin biopsy. The other hereditary sensory neuropathies show different distributions of sensory loss, age of onset and nerve biopsy findings (table 1)7. Hyperpyrexia is the only persistently related cause of death reported and it is noteworthy that our patient has survived through two extremely warm summers. The oldest reported patient is 31; she is wheelchair-bound because of bilateral recurrent hip dislocation and has a Charcot joint of the right shoulder but she continues to perform many activities of daily living8. These patients present to dermatologists or paediatricians with recurrent and non-healing ulcers. Recognition of the diagnosis leads to management of the hyperpyrexia and prevention of its complications. Patients can also be considered for digit-saving measures such as dental clearance but most importantly the family can be given the financial and moral support needed. Damage due to a lack of fear of trauma is understandable but why these children should self-mutilate so progressively is still a mystery.