Idiopathic Pure Sudomotor Failure
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Idiopathic Pure Sudomotor Failure
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 stim ...
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Acquired Idiopathic Generalized Anhidrosis
Acquired idiopathic generalized anhidrosis (AIGA) is characterized by generalized absence of sweating without other autonomic and neurologic dysfunction. Other symptoms include facial flushing, headaches, disorientation, lassitude, hyperthermia, weakness, and palpitations. The diagnosis of acquired idiopathic generalized anhidrosis is made by ruling out other causes of anhidrosis and clinical criteria. Acquired idiopathic generalized anhidrosis is classified into 3 subgroups: idiopathic pure sudomotor failure (IPSF), sweat gland failure (SGF), and sudomotor neuropathy, with each subgroup presenting a different pathogenesis. Treatment includes corticosteroid 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 acti ...
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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 secretion, secretory cells accumulate material at their apical (anatomy), apical ends, and this material then buds off from the cells, forming extracellular vesicle (biology and chemistry), vesicles. The secretory cells therefore lose part of their cytoplasm in the process of secretion. An example of true apocrine glands is the mammary glands, responsible for secreting breast milk. Apocrine glands are also found in the anogenital region and axillae. Apocrine secretion is less damaging to the gland than holocrine secretion (which destroys a cell) but more damaging than merocrine secretion (exocytosis). Apocrine metaplasia Apocrine metaplasia is a reversible transformation of cells to an apocrine phenotype. It is common in the breast in ...
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Hypohidrosis
Hypohidrosis is a disorder in which a person exhibits diminished sweating in response to appropriate stimuli. In contrast with hyp''er''hidrosis, which is a socially troubling yet often benign condition, the consequences of untreated hypohidrosis include hyperthermia, heat stroke and death. An extreme case of hypohidrosis in which there is a complete absence of sweating and the skin is dry is termed anhidrosis. Causes Diagnosis Sweat is readily visualized by a topical indicator such as iodinated starch ( Minor test) or sodium alizarin sulphonate, both of which undergo a dramatic colour change when moistened by sweat. A thermoregulatory sweat test can evaluate the body’s response to a thermal stimulus by inducing sweating through a hot box ⁄ room, thermal blanket or exercise. Failure of the topical indicator to undergo a colour change during thermoregulatory sweat testing indicates hypohidrosis, and further tests may be required to localize the lesion. Magnetic resonance i ...
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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. It is commonly performed by dermatologists. Skin biopsies are also done by family physicians, internists, surgeons, and other specialties. However, performed incorrectly, and without appropriate clinical information, a pathologist's interpretation of a skin biopsy can be severely limited, and therefore doctors and patients may forgo traditional biopsy techniques and instead choose Mohs surgery. There are four main types of skin biopsies: shave biopsy, punch biopsy, excisional biopsy, and incisional biopsy. The choice of the different skin biopsies is dependent on the suspected diagnosis of the skin lesion. Like most biopsies, patient consent and anesthesia (usually lidocaine injected into the skin) are prerequisites. Types Shave biop ...
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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. Parts in the body that use or are affected by acetylcholine are referred to as cholinergic. Substances that increase or decrease the overall activity of the cholinergic system are called cholinergics and anticholinergics, respectively. Acetylcholine is the neurotransmitter used at the neuromuscular junction—in other words, it is the chemical that motor neurons of the nervous system release in order to activate muscles. This property means that drugs that affect cholinergic systems can have very dangerous effects ranging from paralysis to convulsions. Acetylcholine is also a neurotransmitter in the autonomic nervous system, both as an internal transmitter for the sympathetic nervous system and as the final product released by the parasymp ...
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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 on the torso and the extremities. In other mammals, they are relatively sparse, being found mainly on hairless areas such as foot pads. They reach their peak of development in humans, where they may number 200–400/cm2 of skin surface.Bolognia, J., Jorizzo, J., & Schaffer, J. (2012). Dermatology (3rd ed., pp. 539-544). hiladelphia Elsevier Saunders. They produce a clear, odorless substance, sweat, consisting primarily of water. These are present from birth. Their secretory part is present deep inside the dermis. Eccrine glands are composed of an intraepidermal spiral duct, the "acrosyringium"; a dermal duct, consisting of a straight and coiled portion; and a secretory tubule, coiled deep in the dermis or hypodermis. The eccrine gland ope ...
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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 about constriction of the pupil following its dilation. However, due to its side effects it is no longer typically used in the long term management. Onset of effects with the drops is typically within an hour and lasts for up to a day. By mouth it is used for dry mouth as a result of Sjögren syndrome or radiation therapy. Common side effects of the eye drops include irritation of the eye, increased tearing, headache, and blurry vision. Other side effects include allergic reactions and retinal detachment. Use is generally not recommended during pregnancy. Pilocarpine is in the miotics family of medication. It works by activating cholinergic receptors of the muscarinic type which cause the trabecular meshwork to open and the aqueous humor to ...
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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 the body. *Adrenergic nervous system, a part of the autonomic nervous system that uses epinephrine or norepinephrine as its neurotransmitter Regarding proteins: * Adrenergic receptor, a receptor type for epinephrine and norepinephrine; subtypes include α1, α2, β1, β2, and β3 receptors * Adrenergic transporter (norepinephrine transporter), a protein transporting norepinephrine from the synaptic cleft into nerve cells Regarding pharmaceutical drugs: * Adrenergic receptor agonist, a type of drug activating one or more subtypes of adrenergic receptors ** This includes drugs regulating blood pressure and antiasthmatic drugs. * Adrenergic receptor antagonist, a type of drug blocking one or more subtypes of adrenergic receptors ** This ...
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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 vertebrate animal cell. The name "glucocorticoid" is a portmanteau (glucose + cortex + steroid) and is composed from its role in regulation of glucose metabolism, synthesis in the adrenal cortex, and its steroidal structure (see structure below). Glucocorticoids are part of the feedback mechanism in the immune system, which reduces certain aspects of immune function, such as inflammation. They are therefore used in medicine to treat diseases caused by an overactive immune system, such as allergies, asthma, autoimmune diseases, and sepsis. Glucocorticoids have many diverse (pleiotropic) effects, including potentially harmful side effects. They also interfere with some of the abnormal mechanisms in cancer cells, so they are used in high doses to tr ...
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Autonomic Nervous System
The autonomic nervous system (ANS), formerly referred to as the vegetative nervous system, is a division of the peripheral nervous system that supplies viscera, internal organs, smooth muscle and glands. The autonomic nervous system is a control system that acts largely unconsciously and regulates bodily functions, such as the heart rate, its force of contraction, digestion, respiratory rate, pupillary dilation, pupillary response, Micturition, urination, and sexual arousal. This system is the primary mechanism in control of the fight-or-flight response. The autonomic nervous system is regulated by integrated reflexes through the brainstem to the spinal cord and organ (anatomy), organs. Autonomic functions include control of respiration, heart rate, cardiac regulation (the cardiac control center), vasomotor activity (the vasomotor center), and certain reflex, reflex actions such as coughing, sneezing, swallowing and vomiting. Those are then subdivided into other areas and are also ...
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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 many causes, not all of which may be classified as neuropathic. A number of conditions can feature dysautonomia, such as Parkinson's disease, multiple system atrophy, dementia with Lewy bodies, Ehlers-Danlos syndromes, autoimmune autonomic ganglionopathy and autonomic neuropathy, HIV/AIDS, autonomic failure, and postural orthostatic tachycardia syndrome. The diagnosis is achieved through functional testing of the ANS, focusing on the affected organ system. Investigations may be performed to identify underlying disease processes that may have led to the development of symptoms or autonomic neuropathy. Symptomatic treatment is available for many symptoms associated with dysautonomia, and some disease processes can be directly treated. Si ...
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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. Symptoms Cholinergic urticaria typically presents with a number of small, short-lasting hives but may also involve cutaneous inflammation (wheals) and pain which develops usually in response to exercise, bathing, staying in a heated environment, or emotional stress. Although the symptoms subside rapidly, commonly within 1 hour, cholinergic urticaria may significantly impair quality of life, especially in relation to sporting activities. Causes * Sweat hypersensitivity * Acquired anhidrosis and/or hypohidrosis * Idiopathic * Opioid use * Cholinesterase inhibitors Subtypes Sweat hypersensitivity This subtype of CU refers to those who are hypersensitive to their own sweat. Diagnosis Diagnosis is made by injecting autologous (the person's own) ...
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