National Organization for Rare Disorders, Inc.
It is possible that the main title of the report Hyperhidrosis, Primary is not the name you expected.
Related Disorders List
Information on the following diseases can be found in the Related Disorders section of this report:
- Frey's Syndrome
- Greither's Disease
- Secondary Hyperhidrosis (General)
Primary hyperhidrosis is a rare disorder characterized by excessive sweating on the palms of the hands, the soles of the feet, in the armpits (axillary), in the groin area, and/or under the breasts. The exact cause of primary hyperhidrosis is not known. When excessive sweating occurs as part of some other disorder, it is said to be secondary hyperhidrosis, which is a more commonly encountered condition than is primary hyperhidrosis.
The symptoms of primary hyperhidrosis typically begin during childhood or puberty and may often, although not always, persist throughout a person's life. Affected individuals may experience a heightened reaction to certain stimuli that can cause sweating such as anxiety, pain, exercise, tension, caffeine, and/or nicotine. Extreme sweating may occasionally occur all over the body (generalized) or it may be localized in the palms of the hands and the soles of the feet (palmar-plantar hyperhidrosis); the underarm area (axilla); the groin; and under the breasts.
The face may be affected in the form of persistent blushing accompanying the excessive sweating.
When the palms and soles are involved, the skin may develop an abnormal pink or bluish-white appearance. The skin may also become unusually soft (macerated), cracked, or scaly, particularly on the feet.
The exact cause of primary hyperhidrosis is not known. The symptoms of this disorder develop due to overactivity of certain sweat glands, and attacks may be precipitated by social and/or physical stress. Even if stress can be identified as the precipitating cause, the disorder does not appear to be the result of a psychiatric disturbance.
Recently, several papers have been published suggesting a genetic origin and transmission of the disorder as an autosomal dominant trait. However, the gene locus has not been identified.
Chromosomes, which are present in the nucleus of human cells, carry the genetic information for each individual. Human body cells normally have 46 chromosomes. Pairs of human chromosomes are numbered from 1 through 22 and the sex chromosomes are designated X and Y. Males have one X and one Y chromosome, and females have two X chromosomes. Each chromosome has a short arm designated "p" and a long arm designated "q". Chromosomes are further subdivided into many bands that are numbered. For example, "chromosome 11p13" refers to band 13 on the short arm of chromosome 11. The numbered bands specify the location of the thousands of genes that are present on each chromosome.
Genetic diseases are determined by the combination of genes for a particular trait that are on the chromosomes received from the father and the mother.
All individuals carry 4-5 abnormal genes. Parents who are close relatives (consanguineous) have a higher chance than unrelated parents to both carry the same abnormal gene, which increases the risk to have children with a recessive genetic disorder.
Dominant genetic disorders occur when only a single copy of an abnormal gene is necessary for the appearance of the disease. The abnormal gene can be inherited from either parent, or can be the result of a new mutation (gene change) in the affected individual. The risk of passing the abnormal gene from affected parent to offspring is 50% for each pregnancy regardless of the sex of the resulting child.
Primary Hyperhidrosis is a rare disorder that affects males and females in equal numbers. The symptoms of this disorder usually begin during childhood or puberty. Many people with Primary Hyperhidrosis experience relief from the symptoms during adulthood without treatment or obvious reason for the remission.
Secondary Hyperhidrosis is a more common condition that occurs in association with a variety of other disorders. It is important to distinguish Primary Hyperhidrosis from other underlying disorders that can cause excessive sweating (Secondary Hyperhidrosis). These disorders may include impaired thyroid function, malfunction of the pituitary gland, infectious diseases, diabetes, tumors, gout, menopause, side effects of certain drugs, and/or excessive alcohol consumption. (For more information on other disorders associated with profuse sweating, see the Related Disorders section of this report.)
Symptoms of the following disorders can be similar to those of primary hyperhidrosis. Comparisons may be useful for a differential diagnosis:
Secondary hyperhidrosis is a more common condition that occurs in association with a variety of other disorders. It is important to distinguish primary hyperhidrosis from other underlying disorders that can cause excessive sweating (secondary hyperhidrosis). These disorders may include impaired thyroid function, malfunction of the pituitary gland, infectious diseases, diabetes, tumors, gout, menopause, side effects of certain drugs, and/or excessive alcohol consumption.
Frey's Syndrome is a rare neurological disorder that results from injury or surgery near the parotid glands, damaging the facial nerve. The parotid glands are the largest salivary glands in the body and are responsible for the production of saliva. The symptoms of this disorder include flushing and/or sweating on one side of the face. This occurs when hot, spicy, or very acidic foods are consumed. Some affected individuals also experience a temporary lack of sensitivity to heat and burning on the side of the face. The symptoms are usually mild, but may require treatment in some cases. (For more information on this disorder, choose "Frey's" as your search term in the Rare Disease Database.)
Greither's Disease is a rare inherited skin disorder that is characterized by unusual thickness and hardening (keratosis) of the skin on the palms of the hands and soles of the feet. The major symptom of this disorder is excessive sweating of the palms and soles. Areas of hardened skin may extend to the top of the feet and hands. Greither's Disease is inherited as an autosomal dominant genetic trait.
A wide variety of conditions can cause profuse sweating as a symptom. These conditions include, but are not limited to: impaired thyroid function, pituitary abnormalities, infectious diseases, vascular obstruction, myasthenia gravis, diabetes, a variety of tumors, gout, menopause, odontoonychodermal dysplasia, dermatopathia pigmentosa reticularis (DPR), Charcot-Marie-Tooth disease type 1, Ross syndrome, Shapiro's syndrome, Jadassohn-Lewandowsky syndrome, reflex sympathetic dystrophy syndrome, neurogenic acroosteolysis, Weaver syndrome, Gamstorp-Wohlfart syndrome, Meleda disease, Book syndrome, benign essential tremor, blue rubber bleb nevus, pachydermop, and Von Hippel Lindau disease. (For more information on some of these disorders, choose "Myasthenia Gravis," "Charcot Marie Tooth," "Diabetes," "Reflex Sympathetic Dystrophy," "Weaver," "Benign Essential Tremor," "Blue Rubber Bleb Nevus," "Pachydermoperiostosis," and "Von Hippel Lindau" as your search terms in the Rare Disease Database.)
The diagnosis of primary hyperhidrosis is confirmed by thorough clinical history and evaluation that includes the exclusion of other disorders that may cause excessive sweating. A special procedure using infrared imaging techniques may also be effective in confirming the diagnosis of primary hyperhidrosis.
Medical treatments for primary hyperhidrosis should be tried before any decision on surgical treatment is made. Among medical treatments that may be effective in mild or moderate cases are:
People who have mild to moderate primary hyperhidrosis of the palms and soles (Palmar- Plantar type) may find the use of antiperspirants to be effective to some degree. Some physicians recommend aluminum chloride solution 2 to 3 times per week. Wearing cotton socks and canvas shoes and avoiding wool or leather may be of some help as well. The application of medicated powder, formulated to hamper bacterial growth and absorb moisture, may be beneficial in some cases. However, the use of cornstarch is not recommended.
To the palms and/or soles immersed in an appropriate electrolyte solution, a low intensity electric current (15-18 mA) produced by a DC generator is applied. Treatments last for about 20 minutes and take place regularly several times per week. It has been found effective in some patients with light or moderate disease, but some patients complain of the time taken for treatment and/or expense. The treatment is not applicable to patients with hyperhidrosis of the face or thighs.
Mind-altering (psychotropic) drugs as well as anticholinergenic drugs have been tested but the side effects usually prove too severe.
Of very limited effectiveness except in the guise of a placebo effect. Psychiatric treatment may more appropriately be recommended for the consequences of the disorder, which may be acute.
Low-dosage botulinum toxin
In July 2004, the U.S. Food and Drug Administration (FDA) approved botulinum toxin (Botox) as a treatment for primary axillary hyperhidrosis. Used in extremely low dosages, botulinum toxin interferes sufficiently with the action of the chemical nerve impulse transmitter, acetylcholine, which carries the information from one nerve cell to another, to bring some relief from primary hyperhidrosis. The treatment is expensive and must be repeated at regular intervals.
For intractable primary hyperhidrosis, surgery may be the treatment of last resort. In general use are two or three procedures, such as:
Excision of the axillary sweat glands
In cases of axillary hyperhidrosis that are unresponsive to medical treatments, surgery to remove the sweat glands from the armpits may be tried. Scarring may occur subsequent to the procedure, especially if the sweat glands removed were located in the area of the axilla beyond the hairy portion. This procedure does little to reduce sweating in areas other then the armpits.
The nerves controlling the activity of the sweat glands are part of the sympathetic nervous system that, in turn, is part of the larger system over which people have little or no control, the autonomous nervous system. A surgical procedure that interrupts or redirects the activity of these nerves, such as those dealing with sweating, is called a sympathectomy. In cases of extreme hyperhidrosis, sympathectomy by one or another of several surgical techniques, may be selected by the patient. The results of sympathectomy may affect a wide range of sweat glands.
Endoscopic thoracic sympathectomy (ETS)
In contrast to the "open" surgery of the past, ETS requires only two or three small, quarter- to half-inch incisions through which a small telescope and TV camera is passed to locate appropriate nerves and to direct the surgeon's actions. The surgery is performed under general anesthetic and requires the deflation and re-inflation of the lung on each side of the body (bilateral).
A variation of the technique has been reported in children and adolescents whose sympathetic nerve ganglions were clipped with staples rather than cut and excised. The suggested advantage of this technique is that it is reversible.
Patients should be aware of the risks of any surgery using general anesthesia. In addition however, there are side effects of ETS that should be considered. In 50% to 60% of cases, compensatory sweating occurs. The body, in an attempt to make up for the loss of sweating capacity as a result of the surgery, tries to compensate and augment heat-losses by increasing sweating in other locations. In most cases, this is merely a nuisance, but in 5 to 10% of cases, compensatory sweating can be extremely uncomfortable.
Another side effect, in 5 to 10% of cases, is gustatory sweating that occurs during and after eating. Gustatory sweating rarely interferes with a patient's way of life.
If a nerve cluster (ganglion) is damaged during the procedure (1% of cases) a condition known as Horner's syndrome may take place. In Horner's syndrome there may be a small but noticeable droop in one eyelid, an unusually small and narrow pupil in the same eye and a unilateral, dry, non-sweaty side of the face. This condition may be transient or permanent. (For more information on this disorder, choose "Horner" as your search term in the Rare Disease Database.)
Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.
For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:
Tollfree: (800) 411-1222
TTY: (866) 411-1010
For information about clinical trials sponsored by private sources, contact:
Botulinum toxin (Botox) has been approved by the FDA (see Standard Therapies section of this report) for this disorder, but research is continuing on this mode of therapy. The Mt. Sinai Medical Center is sponsoring an interventional study designed to determine the optimal dose of botulinum toxin necessary to decrease sweating in patients with primary hyperhidrosis. For a period of six months, patients will receive injections of botulinum toxin. To assess the efficiency of the drug, patients will undergo an iodine starch test and a test of the sympathetic skin The Mt. Sinai Medical Center is sponsoring an interventional study response. For further information, please contact:
Prof. Horacio Kaufmann, MD
Department of Neurology
Mt. Sinai Medical Center
One Gustave Levy Place
P.O. Box 1052
New York, NY 10029
Phone: (212) 241-7315
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www.parsec.it/summit/hyper 1 e.htm
NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases
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Genetic and Rare Diseases (GARD) Information Center
PO Box 8126
Gaithersburg, MD 20898-8126
International Hyperhidrosis Society
2560 Township Road
Quakertown, PA 18951
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