Alopecia universalis

Información en español Title

Other Names:

Alopecia areata universalis; AU

Categories:

Congenital and Genetic Diseases; Immune System Diseases; Skin Diseases

This disease is grouped under:

Alopecia areata

Summary Summary

Alopecia universalis (AU) is a condition characterized by the complete loss of hair on the scalp and body. It is an advanced form of alopecia areata, a condition that causes round patches of hair loss.^[1] Although the exact cause of AU is unknown, it is thought to be an autoimmune condition in which the person's immune system mistakenly attacks the hair follicles.^[2] An interaction between genetic and environmental factors is thought to play a role in the condition's onset.^[3] There is currently no cure for AU, but sometimes hair regrowth occurs on its own, even after many years.^[2]

Last updated: 9/23/2017

Symptoms Symptoms

AU is characterized by the complete loss of hair on both the scalp and body. Most people with AU do not have other signs and symptoms, but some may experience a burning or itching sensation. In some cases, AU can be associated with other conditions such as atopic dermatitis, thyroid disorders, and/or nail changes (such as pitting).^[4] Anxiety, personality disorders, depression, and paranoid disorders are more common in people with different forms of alopecia areata.^[1]

Last updated: 9/23/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Absent eyebrow	Failure of development of eyebrows	0002223
Absent eyelashes	Failure of development of eyelashes	0000561
Alopecia universalis		0002289
Patchy alopecia	Patchy baldness	0002232
Percent of people who have these symptoms is not available through HPO
Alopecia totalis		0007418
Autoimmunity	Autoimmune disease Autoimmune disorder [ more ]	0002960
Multifactorial inheritance		0001426
Nail pits	Nail pitting Pitted nails [ more ]	0001803
Trachyonychia		0030804

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 11/1/2020

Cause Cause

The exact cause of AU is unknown. AU is an advanced form of alopecia areata (AA), a condition that leads to round patches of hair loss. The most widely accepted hypothesis is that AA is an autoimmune condition in which a person's immune system mistakenly attacks the hair follicles.^[1]

While genetic studies have found that AA and AU are associated with several immune-related genes, they are likely ultimately caused by the interaction of multiple genetic and environmental factors.^[3] This means that even if someone inherits a genetic predisposition to the condition (susceptibility), they may not develop the condition unless something in the environment triggers its onset. However, the exact role of environmental factors is yet to be determined. Factors that may trigger the onset or recurrence of hair loss may include a viral infection, trauma, hormonal changes, and emotional or physical stress.^[5]

Last updated: 9/23/2017

Inheritance Inheritance

AU is believed to be a multifactorial condition, which means it is likely caused by a combination of environmental triggers and genetic predisposition (susceptibility).^[2] While a predisposition can be inherited and some people with AU have a family history, the condition itself is not thought to be inherited.

Last updated: 9/23/2017

Diagnosis Diagnosis

A diagnosis of AU is usually based on the signs and symptoms present. In rare cases, a scalp biopsy may be needed to confirm the diagnosis.^[1]

Last updated: 9/23/2017

Treatment Treatment

No therapy has been found to work for everyone who has alopecia universalis (AU) which makes managing AU challenging. Although multiple treatments have been explored, no therapy is currently approved by the United States Food and Drug Administration (FDA). Some promising therapies include:^[6]

diphenylcyclopropenone (DPCP)
squaric acid dibutylester (SADBE)
photodynamic therapy
steroids
cyclosporine in combination with methylprednisolone (a steroid)

There are several recent studies showing that a class of medication known as JAK inhibitors, which includes Tofacitinib and Ruxolitinib, are effective in alopecia areata, including AU. However, JAK inhibitors have not yet been approved by the FDA for use in skin conditions.^[7]^[8]

In some people with AU, hair regrowth occurs without treatment, sometimes after many years.^[9]

There are steps that can be taken to decrease the chance of getting too much sun and minimize other discomforts related to having no hair. These may include:^[2]

Using sunscreen on the scalp, face, and all areas of the skin exposed to the sun
Wearing eyeglasses or sunglasses to protect the eyes from too much sun and from dust and debris when eyebrows or eyelashes are missing
Wearing wigs, caps, or scarves to protect the scalp from the sun and keep the head warm
Applying an ointment inside the nostrils to keep them moisturized and help to protect against organisms invading the nose when nostril hair is missing

Many other treatments have been reported to have variable response rates in small studies in alopecia areata. These include latanoprost, nitrogen mustard, massage and relaxation, isoprinosine, acupuncture, and aromatherapy, among others.^[8]

Last updated: 9/23/2017

Prognosis Prognosis

The course of AU is highly unpredictable, and this uncertainty is one of the most difficult and frustrating aspects of the disease. People with AU may continue to lose hair, or hair loss may stop. Hair that has already been lost may or may not grow back.^[2] It has been estimated that only about 10% of patients experience full recovery.^[4]

Last updated: 9/23/2017

Statistics Statistics

According to Orphanet's Report Series on the prevalence and incidence of rare diseases from 2017, the estimated prevalence of alopecia universalis, based on European data, is 25/100,000 people (which is approximately 1 in 4000 people).^[10] We are not aware of statistics specific to the United States.

To our knowledge, estimates of the number of people with alopecia areata who eventually develop alopecia unversalis or totalis range from 7% to 25%.^[1]^[11]

Last updated: 10/3/2017

Do you have updated information on this disease? We want to hear from you.

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Alopecia universalis. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.

Patient Registry

A registry supports research by collecting of information about patients that share something in common, such as being diagnosed with Alopecia universalis. The type of data collected can vary from registry to registry and is based on the goals and purpose of that registry. Some registries collect contact information while others collect more detailed medical information. Learn more about registries.

Registries for Alopecia universalis:
Alopecia Areata Registry

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Hair Loss Association
23679 Calabasas Road # 682
Calabasas, CA 91301-1502
E-mail: info-ahla@americanhairloss.org
Website: http://americanhairloss.org
Children's Alopecia Project, Inc.
906 Penn Avenue 1st Floor
Wyomissing, PA 19610
Telephone: (610) 468-1011
E-mail: Jeff.Woytovich@ChildrensAlopeciaProject.org
Website: https://childrensalopeciaproject.org
National Alopecia Areata Foundation (NAAF)
65 Mitchell Boulevard
Suite 200-B
San Rafael, CA 94903
Telephone: +1-415-472-3780
Fax: +1-415-472-1800
E-mail: info@naaf.org
Website: https://www.naaf.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.
MedlinePlus was designed by the National Library of Medicine to help you research your health questions, and it provides more information about this topic.
The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS) support research into the causes, treatment, and prevention of arthritis and musculoskeletal and skin diseases, the training of basic and clinical scientists to carry out this research, and the dissemination of information on research progress in these diseases. Click on the link to view information on this topic.
The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Alopecia universalis. Click on the link to view a sample search on this topic.

Resources for Kids

The American Hair Loss Association Web site lists resources for kids with alopecia. Click on American Hair Loss Association to view the page.

News & Events News & Events

News

Rare Diseases Are Not Rare - Gallery of Creative Work Raises Awareness of Rare Diseases
September 1, 2020
NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community
May 22, 2020
NCATS Translational Approach Addresses COVID-19
May 21, 2020

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know.

Have a question? Contact a GARD Information Specialist.

References References

Bolduc C. Alopecia Areata. Medscape Reference. May 8, 2017; http://emedicine.medscape.com/article/1069931-overview.
Questions and Answers about Alopecia Areata. NIAMS. May, 2016; https://www.niams.nih.gov/Health_Info/Alopecia_Areata/default.asp.
Lee S., et. al. Exomic sequencing of immune-related genes reveals novel candidate variants associated with alopecia universalis. PLoS One. 2013; 8(1):
Cho HH, Jo SJ, Paik SH, Jeon HC, Kim KH, Eun HC, Kwon OS. Clinical characteristics and prognostic factors in early-onset alopecia totalis and alopecia universalis. J Korean Med Sci. July, 2012; 27(7):799-802. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3390731/.
Oakley A. Alopecia areata. DermNet NZ. December, 2015; http://www.dermnetnz.org/topics/alopecia-areata/.
Kassira S, Korta DZ, W Chapman L, Dann F. Review of treatment for alopecia totalis and alopecia universalis. Int J Dermatol. April 5, 2017; [Epub ahead of print]:https://www.ncbi.nlm.nih.gov/pubmed/28378336.
Frellick M. JAK Inhibitors Show Promise for Alopecia, Eczema, Vitiligo. American Academy of Dermatology (AAD) Annual Meeting. . Disponible en:. March 07, 2017; http://www.medscape.com/viewarticle/876855.
Bolduc C. Alopecia Areata Treatment & Management. Medscape Reference. May 8, 2017; http://emedicine.medscape.com/article/1069931-treatment#d11.
Treatments for Alopecia Areata. National Alopecia Areata Foundation. 2017; http://www.naaf.org/site/PageServer?pagename=about_alopecia_treatment.
Prevalence and incidence of rare diseases: Bibliographic data. Orphanet Report Series. June, 2017; 1:7. http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_alphabetical_list.pdf.
Pratt CH, King, Jr. LE, Messenger AG, Christiano AM, Sundberg JP. Alopecia areata. Nat Rev Dis Primers. March 16, 2017; 3:https://www.nature.com/articles/nrdp201711.
Nucara S, Colao E, Mangone G, et. al. Identification of a new mutation in the gene coding for hairless protein responsible for alopecia universalis: The importance of direct gene sequencing. Dermatology Online J. January 15, 2011; 17(1):3. http://www.ncbi.nlm.nih.gov/pubmed/21272494.