$Unique_ID{BRK03442} $Pretitle{} $Title{Alopecia Areata} $Subject{Alopecia Areata Alopecia Celsi Alopecia Circumscripta Jonston's Alopecia Porrigo Decalvans Vitiligo Capitis Cazenave's Vitiligo Celsus' Vitiligo Alopecia Universalis (Alopecia Totalis) Alopecia Semiuniversalis Trichotillomania Hypotrichiasis (Alopecia Congenitalis, Alopecia Adnata, Congenital Baldness, Congenital Alopecia) Alopecia Medicamentosa Alopecia Mucinosa Alopecia Neurotica Postpartum Alopecia Premature Alopecia Alopecia Presenilis Alopecia Symptomatica Syphilitic Alopecia Alopecia Toxica (Toxic Alopecia) Alopecia Triangularis Congenitalis} $Volume{} $Log{} Copyright (C) 1987, 1988, 1989 National Organization for Rare Disorders, Inc. 443: Alopecia Areata ** IMPORTANT ** It is possible the main title of the article (Alopecia Areata) is not the name you expected. Please check the SYNONYMS listing on the next page to find alternate names, disorder subdivisions, and related disorders covered by this article. Synonyms Alopecia Celsi Alopecia Circumscripta Jonston's Alopecia Porrigo Decalvans Vitiligo Capitis Cazenave's Vitiligo Celsus' Vitiligo DISORDER SUBDIVISIONS Alopecia Universalis (Alopecia Totalis) Alopecia Semiuniversalis Information on the following diseases can be found in the Related Disorders section of this report: Trichotillomania Hypotrichiasis (Alopecia Congenitalis, Alopecia Adnata, Congenital Baldness, Congenital Alopecia) Alopecia Medicamentosa Alopecia Mucinosa Alopecia Neurotica Postpartum Alopecia Premature Alopecia Alopecia Presenilis Alopecia Symptomatica Syphilitic Alopecia Alopecia Toxica (Toxic Alopecia) Alopecia Triangularis Congenitalis General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources section of this report. Alopecia Areata is a progressive condition characterized by loss of hair. The cause is unknown, and unpredictable hair loss is the only noticeable symptom. Regrowth of hair may or may not occur. Some cases may be associated with endocrine abnormalities, infection of the scalp, or an autoimmune reaction. Hair loss is usually confined to the head and face, although the entire body may be involved. Symptoms Alopecia Areata often begins suddenly with oval or round bald patches appearing most commonly on the scalp. Other areas of hairy skin may also be involved. Gradually, the affected skin becomes white and smooth. New patches may spread by joining existing bald patches. These larger bald areas can appear while hair is regrowing in older hairless patches. Loss of hair can be permanent in some cases. Hair follicles may deteriorate, but oil producing glands in the skin (sebaceous glands) usually change very little. The skin does not become hard or atrophied. In a very few cases, all body hair may be lost. Cases beginning during childhood tend to be more severe than cases with an onset during adulthood. Causes The exact cause of Alopecia Areata is not known. An autoimmune mechanism is suspected in this disorder. Autoimmune disorders are caused when the body's natural defenses (antibodies) against invading organisms suddenly begin to attack healthy tissue. Some cases may be linked to abnormal reactions by blood cells (serum antibodies) to a thyroid protein (thyroglobulin), stomach (parietal) cells, or adrenal cells. Affected Population Alopecia Areata affects males and females in equal numbers. Cases may begin during childhood or adulthood. According to a 1983 study at the Mayo Clinic in Rochester, MN, approximately 2 million cases of this disorder were estimated in the United States at that time. Related Disorders Loss of hair (alopecia) can occur from a wide variety of causes. Symptoms of the following disorders can be similar to those of Alopecia Areata. Comparisons may be useful for a differential diagnosis: Trichotillomania, also known as hair pulling, is a neurotic habit that usually appears in children. It may remain undiagnosed for a long time. The hairs may be broken off or pulled out. Stubby regrowth may replace damaged hair or bald areas. This mental illness may be hard to distinguish from Alopecia Areata without careful observation of the affected child's habits. Hair can be similarly damaged by permanent wave solutions, softeners or hot combs. (For more information on this disorder, choose "trichotillomania" as your search term in the Rare Disease Database.) Hypotrichiasis (Hypotrichosis, Alopecia Congenitalis, Alopecia Adnata, Congenital Alopecia, Congenital Baldness) is a condition characterized by the absence of hair at birth. This disorder is usually inherited as a dominant trait, but can also be due to a recessive gene. It often occurs in association with other surface skin layer (ectodermal) defects. Alopecia Medicamentosa is characterized by widespread hair loss, most commonly of the scalp, caused by a reaction to various types of drugs in sensitive or allergic individuals. It may also be a result of chemotherapy used in treating various disorders (e.g., cancer). Alopecia Mucinosa, also known as Follicular Mucinosis, occurs in children and young adults. Hard, reddish, well defined plaques underlie the areas of hair loss. A fine scaling may develop on the face, scalp, trunk, arms or legs. A loss of sensation may occur as the plaques develop. The exact cause of this form of hair loss in not known, although a skin inflammation is suspected. Symptoms often spontaneously resolve after a few months in many cases. Alopecia Neurotica is characterized by hair loss caused by injury to the nerves in the area where balding occurs. Postpartum Alopecia is characterized by temporary loss of hair at the termination of a pregnancy. The cause is not known. Premature Alopecia is characterized by male pattern baldness occurring at an abnormally early age. Alopecia Presenilis is characterized by ordinary or common baldness occurring in early or middle life without any apparent disease of the scalp. This condition is very common in males, but rare in females. Alopecia Symptomatica is characterized by hair loss associated with other illnesses or conditions, most commonly following prolonged illnesses marked by high fever. Alopecia Toxica, also known as Toxic Alopecia, is characterized by hair loss thought to be caused only by fever. Alopecia Triangularis Congenitalis is a congenital defect consisting of a triangular patch of baldness on the front of the scalp. Therapies: Standard Treatment of Alopecia Areata is directed at producing regrowth of hair. Drugs such as systemic corticosteroids may cause the hair to grow, but long- term treatment may have undesirable side effects. Triamcinolone acetonide suspension may be beneficial but the effect is often temporary. For cosmetic reasons, wigs and hairpieces may be necessary, especially for affected women and children. Therapies: Investigational Treatment of Alopecia Areata involving a combination of 8-methoxypsoralen ointment and ultraviolet light exposure is being attempted experimentally, and may not be successful in all cases. More research is necessary to determine other treatment combinations to prolong beneficial effects in patients. Some researchers believe ultraviolet light may trigger a change in the immune system (immunomodulation) which might control hair loss in Alopecia Areata. Synthetic immunomodulator drugs such as Isoprinosine and diphencyprone are also under investigation as possible treatments for this disorder. A clinical trial involving low doses of the drug spironolactone, also used to treat excessive hair growth in females due to hormonal imbalances, is under way. A trial of the drug minoxidil indicated no benefit for severe Alopecia Areata patients. Other tests involve the drug dinitrochlorobenzene (in ointment form) as compared with squaric acid dibutylester. This is not recommended due to adverse side effects in some test participants. More extensive research is necessary before the therapeutic value of these drugs can be evaluated. Cyclosporine (Sandimmune) may be of potential benefit for treating a number of dermatologic diseases. These include Pemphigus and Bullous Pemphigoid, Posterior Uveitis and Behcet's Syndrome, collagen vascular disorders such as severe Dermatomyositis, Sjogren's Syndrome, and Scleroderma, Mycosis Fungoides, and Alopecia Areata. However, this drug may also be associated with severe and life-threatening side effects which would limit its use in many patients. Careful monitoring of this drug by a physician is necessary to guard against possible toxic side effects. Relapses can occur when the drug is discontinued. More research is needed before cyclosporine can be recommended as a treatment for all but the most severe cases of the disorders listed above. Even for the most severe cases its use is still experimental, and long-term effects are unknown. This disease entry is based upon medical information available through November 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Alopecia Areata, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 National Alopecia Areata Foundation P.O. Box 15076 San Rafael, CA 94901-0760 (415) 456-4644 The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 References TOPICAL PHOTOCHEMOTHERAPY FOR ALOPECIA AREATA: A.J. Mitchell, et al.; J Am Acad Dermatol (April 1985, issue 12(4) ). Pp. 644-649. CLINICAL AND IMMUNOLOGIC RESPONSE TO ISOPRINOSINE IN ALOPECIA AREATA AND ALOPECIA UNIVERSALIS: ASSOCIATION WITH AUTOANTIBODIES: M. Lowy, et al.; J Am Acad Dermatol (January 1985, issue 12(1 Pt 1). Pp. 78-84. LOW-DOSE SPIROMOLACTONE IN THE TREATMENT OF FEMALE HIRSUTISM: Int J Fertil (Jan-Feb 1987, issue 32(1)). Pp. 41-45.