Congenital Insensitivity to Pain with Anhidrosis (CIPA) is a rare disorder that affects the afferent nervous system responsible for pain and temperature.


Chromosome location: 1q23.1

Gene affected: NTRK1 (receptor tyrosine kinase for nerve growth factor NGF)


Inheritance: autosomal recessive


Key clinical features:

(1) insensitivity to pain

(2) defective temperature sensation

(3) anhidrosis with fever and heat intolerance


Variable findings:

(1) self-mutilating behavior

(2) immunodeficiency with reduced immunoglobulins and recurrent infections

(3) mental retardation


Affected patients fail a number of pharmacologic stimulation tests:

(1) lacrimation following subcutaneous injection of neostigmine

(2) unresponsiveness to mehacholine

(3) absence of a flare response to histamine

(4) absence of sweating following any normal stimulus


A skin biopsy shows histologically normal sweat glands.


Naloxone therapy can be effective in these patients (Yanagida, 1978)


To read more or access our algorithms and calculators, please log in or register.