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 Somatic may refer to: * Somatic (biology), referring to the cells of the body in contrast to the germ line cells ** Somatic cell, a non-gametic cell in a multicellular organism * Somatic nervous system, the portion of the vertebrate nervous sys ...

nervous dysfunctions and without persistent organic cutaneous

lesions A lesion is any damage or abnormal change in the tissue of an organism, usually caused by disease or trauma. ''Lesion'' is derived from the Latin "injury". Lesions may occur in plants as well as animals. Types There is no designated classifi ...

. The term IPSF was first introduced in 1994 after researchers at

Saitama Medical School is a private university at Moroyama, Saitama is a town located in Saitama Prefecture, Japan. , the town had an estimated population of 33,178 in 15,888 households and a population density of 970 persons per km². The total area of the town is . ...

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 Sudomotor function refers to the autonomic nervous system control of sweat gland activity in response to various environmental and individual factors. Sweat production is a vital thermoregulatory mechanism used by the body to prevent heat-related ...

neuropathy Peripheral neuropathy, often shortened to neuropathy, is a general term describing disease affecting the peripheral nerves, meaning nerves beyond the brain and spinal cord. Damage to peripheral nerves may impair sensation, movement, gland, or o ...

or sweat gland failure.

Clinical features

* Early onset in life * Acute or sudden onset * Concomitant sharp pain or

cholinergic urticaria Cholinergic urticaria (CU) presents with tiny very itchy wheals and small bumps on a reddish background. It is a type of physical urticaria (or ''hives'') that appears when a person is sweating or their core body temperature increases. Sympt ...

over the entire body * Absence of other

autonomic dysfunction Dysautonomia or autonomic dysfunction is a condition in which the autonomic nervous system (ANS) does not work properly. This may affect the functioning of the heart, bladder, intestines, sweat glands, pupils, and blood vessels. Dysautonomia has ...

* Elevated serum

IgE Immunoglobulin E (IgE) is a type of antibody (or immunoglobulin (Ig) " isotype") that has been found only in mammals. IgE is synthesised by plasma cells. Monomers of IgE consist of two heavy chains (ε chain) and two light chains, with the ε c ...

levels * Marked response to

glucocorticoids Glucocorticoids (or, less commonly, glucocorticosteroids) are a class of corticosteroids, which are a class of steroid hormones. Glucocorticoids are corticosteroids that bind to the glucocorticoid receptor that is present in almost every vertebr ...

* Preserved

apocrine Apocrine () glands are a type of exocrine gland, which are themselves a type of gland, i.e. a group of cells specialized for the release of secretions. Exocrine glands secrete by one of three means: holocrine, merocrine and apocrine. In apocrine ...

sweating (

adrenergic Adrenergic means "working on adrenaline (epinephrine) or noradrenaline (norepinephrine)" (or on their receptors). When not further qualified, it is usually used in the sense of enhancing or mimicking the effects of epinephrine and norepinephrine in ...

innervation)

Pathology

Intracutaneous injection of

pilocarpine Pilocarpine is a medication used to reduce pressure inside the eye and treat dry mouth. As eye drops it is used to manage angle closure glaucoma until surgery can be performed, ocular hypertension, primary open angle glaucoma, and to bring abou ...

(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 Eccrine sweat glands (; from Greek ''ekkrinein'' 'secrete'; sometimes called merocrine glands) are the major sweat glands of the human body, found in virtually all skin, with the highest density in palm and soles, then on the head, but much less ...

. * Interference in

acetylcholine Acetylcholine (ACh) is an organic chemical that functions in the brain and body of many types of animals (including humans) as a neurotransmitter. Its name is derived from its chemical structure: it is an ester of acetic acid and choline. Part ...

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. IPSF is characterized by the absence of sweating on the palms and soles, along with cholinergic urticaria. Skin biopsy and specific markers can help distinguish IPSF from other sweat-related conditions.

Management

Treatment of AIGA almost always consists of steroid pulse therapy or high-dose oral steroids and is not consistently effective. Much remains unclear regarding the reasons for recurrent anhidrosis.

Epidemiology

The overwhelming majority of reported AIGA patients are Japanese, but whether AIGA is truly rare in whites or has been simply underreported by Western physicians remains unclear. AIGA is most prevalent among young men. In a 64 case review of the literature 58 reported cases were males and 6 female, with a mean age of onset at 28 ± 11 years. Cholinergic urticaria or sharp pain over the entire body induced by elevated body temperature was reported in 32 cases (50%). Of 28 cases tested, 12 (43%) displayed elevated serum IgE levels.

Skin biopsy Skin biopsy is a biopsy technique in which a skin lesion is removed to be sent to a pathologist to render a microscopic diagnosis. It is usually done under local anesthetic in a physician's office, and results are often available in 4 to 10 days. ...

was performed in 53 cases, with normal findings in 20 cases (38%), and cellular infiltrates in sweat glands or ducts in 23 cases (43%).

References

External links

{{Medical resources , DiseasesDB = 21064 , ICD10 = {{ICD10, L, 74, 8, l, 60 , ICD9 = {{ICD9, 705.0 Conditions of the skin appendages Syndromes