Acquired idiopathic generalized anhidrosis (AIGA) is characterized by generalized absence of

sweating Perspiration, also known as sweat, is the fluid secreted by sweat glands in the skin of mammals. Two types of sweat glands can be found in humans: eccrine glands and apocrine glands. The eccrine sweat glands are distributed over much of the ...

without other autonomic and neurologic dysfunction. Other symptoms include

facial flushing Flushing is to become markedly red in the face and often other areas of the skin, from various physiological conditions. Flushing is generally distinguished from blushing, since blushing is psychosomatic, milder, generally restricted to the face ...

headaches A headache, also known as cephalalgia, is the symptom of pain in the face, head, or neck. It can occur as a migraine, tension-type headache, or cluster headache. There is an increased risk of depression in those with severe headaches. Head ...

disorientation Orientation is a function of the mind involving awareness of three dimensions: time, place and person. Problems with orientation lead to ''dis''orientation, and can be due to various conditions. It ranges from an inability to coherently understand ...

lassitude Fatigue is a state of tiredness (which is not sleepiness), exhaustion or loss of energy. It is a symptom of any of various diseases; it is not a disease in itself. Fatigue (in the medical sense) is sometimes associated with medical conditions ...

hyperthermia Hyperthermia, also known as overheating, is a condition in which an individual's body temperature is elevated beyond normal due to failed thermoregulation. The person's body produces or absorbs more heat than it dissipates. When extreme te ...

weakness Weakness is a symptom of many different medical conditions. The causes are many and can be divided into conditions that have true or perceived muscle weakness. True muscle weakness is a primary symptom of a variety of skeletal muscle diseases, ...

, and

palpitations Palpitations occur when a person becomes aware of their heartbeat. The heartbeat may feel hard, fast, or uneven in their chest. Symptoms include a very fast or irregular heartbeat. Palpitations are a sensory symptom. They are often described as ...

. The diagnosis of acquired idiopathic generalized anhidrosis is made by ruling out other causes of

anhidrosis Hypohidrosis is a medical condition in which a person exhibits diminished sweating in response to appropriate stimuli. In contrast with hyperhidrosis, which is a socially troubling yet often benign condition, the consequences of untreated hypohidr ...

and clinical criteria. Acquired idiopathic generalized anhidrosis is classified into 3 subgroups: idiopathic pure sudomotor failure (IPSF),

sweat gland Sweat glands, also known as sudoriferous or sudoriparous glands, , are small tubular structures of the skin that produce sweat. Sweat glands are a type of exocrine gland, which are glands that produce and secrete substances onto an epithelial s ...

failure (SGF), and sudomotor

neuropathy Peripheral neuropathy, often shortened to neuropathy, refers to damage or disease affecting the nerves. Damage to nerves may impair sensation, movement, gland function, and/or organ function depending on which nerve fibers are affected. Neuropa ...

, with each subgroup presenting a different

pathogenesis In pathology, pathogenesis is the process by which a disease or disorder develops. It can include factors which contribute not only to the onset of the disease or disorder, but also to its progression and maintenance. The word comes . Descript ...

. Treatment includes

corticosteroid Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invo ...

therapy. Acquired idiopathic generalized anhidrosis is considered to be rare but the exact prevalence is unknown. It is more common in males than females and usually manifests during the second and fourth decades of life.

Signs and symptoms

When exposed to heat stimuli like high temperatures, high humidity, or physical activity, patients with AIGA are unable to sweat appropriately. Typically,

and

hypohidrosis Hypohidrosis is a medical condition in which a person exhibits diminished sweating in response to appropriate stimuli. In contrast with hyperhidrosis, which is a socially troubling yet often benign condition, the consequences of untreated hypohidr ...

are distributed symmetrically across the trunk. It is uncommon for the palms, soles, or axillae to be afflicted, although it can also affect the face and the extremities. These patients are unable to sweat, which is crucial for controlling

body temperature Thermoregulation is the ability of an organism to keep its body temperature within certain boundaries, even when the surrounding temperature is very different. A thermoconforming organism, by contrast, simply adopts the surrounding temperature ...

. As a result, heat builds up during physical activity or in hot conditions. Such individuals may exhibit a variety of symptoms, including

, and

. Certain patients may get

heatstroke Heat stroke or heatstroke, also known as sun-stroke, is a severe heat illness that results in a body temperature greater than , along with red skin, headache, dizziness, and confusion. Sweating is generally present in exertional heatstroke, ...

. In addition,

cholinergic urticaria Cholinergic urticaria (CholU, CU) is a form of hives (urticaria) that is triggered by an elevation in body temperature, breaking a sweat, or exposure to heat. It is also sometimes called exercise-induced urticaria or heat hives. The condition is ...

's prickling pain and rash are commonly seen. These symptoms frequently have a chronic course, while they occasionally resolve spontaneously.

Causes

Acquired idiopathic generalized anhidrosis appears to have a variety of etiologies. Theoretically, dysfunction or degeneration of

cholinergic Cholinergic agents are compounds which mimic the action of acetylcholine and/or butyrylcholine. In general, the word " choline" describes the various quaternary ammonium salts containing the ''N'',''N'',''N''-trimethylethanolammonium cation ...

sympathetic

nerve fibers A nerve is an enclosed, cable-like bundle of nerve fibers (called axons). Nerves have historically been considered the basic units of the peripheral nervous system. A nerve provides a common pathway for the electrochemical nerve impulses called ...

involved in sweating (sudomotor

), dysfunction of

acetylcholine receptors An acetylcholine receptor (abbreviated AChR) or a cholinergic receptor is an integral membrane protein that responds to the binding of acetylcholine, a neurotransmitter. Classification Like other transmembrane receptors, acetylcholine receptors ...

and/or

signals ( idiopathic pure sudomotor failure may fall under this category), and primary failures of the

sweat glands Sweat glands, also known as sudoriferous or sudoriparous glands, , are small tubular structures of the skin that produce sweat. Sweat glands are a type of exocrine gland, which are glands that produce and secrete substances onto an epithelial sur ...

with apparent morphological changes of the sweat apparatus can all be taken into consideration.

Diagnosis

Congenital conditions such

Fabry disease Fabry disease, also known as Anderson–Fabry disease, is a rare genetic disease that can affect many parts of the body, including the kidneys, heart, brain, and skin. Fabry disease is one of a group of conditions known as lysosomal storage dis ...

, hypohidrotic/ anhidrotic ectodermal dysplasia, and

congenital insensitivity to pain with anhidrosis 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 coincide ...

should be ruled out in order to diagnose acquired idiopathic generalized anhidrosis is made. It's also necessary to rule out secondary sweating disorders linked to neurological conditions, metabolic illnesses, Sjögren's syndrome, and drug-induced sweating irregularities. The diagnostic criteria for acquired idiopathic generalized anhidrosis is as follows: # Despite the widespread distribution of idiopathic

lesions in a non-segmental spinal pattern, no additional neurological or autonomic symptoms are noted. # At least 25% of the body is affected by anhidrotic or hypohidrotic regions. These are characterized as regions that do not become black when subjected to the thermoregulatory sweat test (which is based on the Minor method utilizing the

iodine Iodine is a chemical element; it has symbol I and atomic number 53. The heaviest of the stable halogens, it exists at standard conditions as a semi-lustrous, non-metallic solid that melts to form a deep violet liquid at , and boils to a vi ...

-starch reaction) and other techniques, or regions that thermographically reveal

. When criteria A and B are met, the diagnosis of acquired idiopathic generalized anhidrosis is made. Quantitative sudomotor axon reflex test and

microneurography Microneurography is a neurophysiological method employed to visualize and record the traffic of nerve impulses that are conducted in peripheral nerves of waking human subjects. It can also be used in animal recordings. The method has been successf ...

are used in the diagnosis of acquired idiopathic generalized anhidrosis. However, these refined methods are mostly used for research purposes and not generally available.

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 day ...

analysis may play a crucial role in the identification of acquired idiopathic generalized anhidrosis subgroups.

Treatment

The early phases of acquired idiopathic generalized anhidrosis are indicated for

therapy.

Corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...

are said to have a negative effect on patients who have delayed starting treatment or who have

tissue deterioration. In addition,

antihistamines Antihistamines are drugs which treat allergic rhinitis, common cold, influenza, and other allergies. Typically, people take antihistamines as an inexpensive, generic (not patented) drug that can be bought without a prescription and provides r ...

cyclosporine Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease, nephrotic syndrome, ecz ...

, and gabapentine have been used to treat acquired idiopathic generalized anhidrosis.

Epidemiology

Since there are currently no published epidemiological data on acquired idiopathic generalized anhidrosis, its prevalence and morbidity are unclear. Given that as of 2016, there had only been about 100 cases reported, acquired idiopathic generalized anhidrosis is thought to be extremely uncommon. Most known cases of acquired idiopathic generalized anhidrosis were documented in

Japan Japan is an island country in East Asia. Located in the Pacific Ocean off the northeast coast of the Asia, Asian mainland, it is bordered on the west by the Sea of Japan and extends from the Sea of Okhotsk in the north to the East China Sea ...

. Therefore, it is uncertain if its prevalence changes according to area and race. With men making up over 80% of documented instances, acquired idiopathic generalized anhidrosis is noticeably more common in this population. acquired idiopathic generalized anhidrosis can strike at any age, from infancy to the eighth decade of life, even though the average onset age falls between the second and fourth decades of life.

References

External links

{{Disorders of skin appendages Conditions of the skin appendages