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1960: Ervin and Sternbach describe 6 members from a 2-generation family with dominantly-inherited congenital insensitivity to pain. [ 4 ] 1974: Comings and Amromin describe 3 members from a 2-generation family which consisted of a mother, her son and her daughter with the symptoms characteristic of Marsili syndrome, there was a possibility that ...
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder of the nervous system which prevents the feeling of pain or temperature and prevents a person from sweating. Cognitive disorders are commonly coincidental.
The diagnostic criteria for acquired idiopathic generalized anhidrosis is as follows: [6] Despite the widespread distribution of idiopathic anhidrosis or hypohidrosis lesions in a non-segmental spinal pattern, no additional neurological or autonomic symptoms are noted. [6] At least 25% of the body is affected by anhidrotic or hypohidrotic regions.
Type 4, congenital insensitivity to pain with anhidrosis (CIPA), is an autosomal recessive condition and affected infants present with episodes of hyperthermia unrelated to environmental temperature, anhidrosis and insensitivity to pain. Palmar skin is thickened and charcot joints are commonly present.
Idiopathic pure sudomotor failure (IPSF) is the most common cause of a rare disorder known as acquired idiopathic generalized anhidrosis (AIGA), a clinical syndrome characterized by generalized decrease or absence of sweating without other autonomic and somatic nervous dysfunctions and without persistent organic cutaneous lesions.
The treatment options for hypohidrosis and anhidrosis are largely limited to preventing overheating, and attempting to resolve or prevent further deterioration of any known underlying causes. Those with hypohidrosis should avoid drugs that can aggravate the condition (see "Medications", under § Causes). They should limit activities that raise ...
Insensitivity to pain means that the painful stimulus is not even perceived: a patient cannot describe the intensity or type of pain. Indifference to pain means that the patient can perceive the stimulus, but lacks an appropriate response: they do not flinch or withdraw when exposed to pain.
Little to no ability to sweat (hypo/anhidrosis) Frontal bossing (prominence of the forehead) Wrinkling under the eyes; Periorbital hyperpigmentation; These symptoms are accompanied by an immunodeficiency that affects the entire body and impairs the body's antibody response (especially that to polysaccharides).