Idiopathic pure sudomotor failure
medical condition
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 term IPSF was first introduced in 1994 after researchers at Saitama Medical School speculated the primary lesion sites in patients were within cholinergic receptors of the sweat glands. The term IPSF represent a distinct subgroup of AIGA without sudomotor neuropathy or sweat gland failure.
Clinical features
Early onset in life
Acute or sudden onset
Concomitant sharp pain or cholinergic urticaria over the entire body
Absence of other autonomic dysfunction
Elevated serum IgE levels
Marked response to glucocorticoids
Preserved apocrine sweating (adrenergic innervation)
Pathology
Intracutaneous injection of pilocarpine (sweat gland stimulant) is known to evoke no sweat response, indicating that lesions are on the post-synaptic side of the nerve-sweat gland junction.
The proposed pathomechanisms of idiopathic pure sudomotor failure include:
A deficit within muscarinic cholinergic receptors of the eccrine sweat glands.
Interference in acetylcholine transmission to cholinergic receptors.
A cross-reactive immune response which interferes with cholinergic transmission in the eccrine glands.
Components of an immediate-type allergy (based on the dramatic resumption of axon reflex sweating following glucocorticoid treatment).
Diagnosis
IPSF is a diagnosis made after ruling out other possible causes. It involves a lack of sweating all over the body, excluding certain congenital and acquired conditions.
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This entry incorporates text from “Idiopathic pure sudomotor failure” on English Wikipedia. Contributors are listed in the page history. Text is available under the Creative Commons Attribution-ShareAlike 4.0 License. Selected authority identifiers and statements are retrieved from Wikidata under CC0; their references and qualifiers remain part of the verification path.