Alopecia universalis

Title

Other Names:

Alopecia areata universalis; AU

Categories:

Congenital and Genetic Diseases; Immune System Diseases; Skin Diseases

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 an affected 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: 5/17/2017

Symptoms Symptoms

Alopecia universalis (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: 5/17/2017

The Human Phenotype Ontology (HPO) provides the following list of features that have been reported in people with this condition. Much of the information in the HPO comes from Orphanet, a European rare disease database. If available, the list includes a rough estimate of how common a feature is (its frequency). Frequencies are based on a specific study and may not be representative of all studies. You can use the MedlinePlus Medical Dictionary for definitions of the terms below.

Signs and Symptoms	Approximate number of patients (when available)
Absent eyebrow	Very frequent (present in 80%-99% of cases)
Absent eyelashes	Very frequent (present in 80%-99% of cases)
Alopecia areata	Very frequent (present in 80%-99% of cases)
Alopecia universalis	Very frequent (present in 80%-99% of cases)
Alopecia totalis	-
Autoimmunity	-
Multifactorial inheritance	-
Nail pits	-

Last updated: 9/1/2017

Cause Cause

The exact cause of alopecia universalis (AU) is not known. 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 an affected 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. Proposed 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: 5/17/2017

Inheritance Inheritance

Alopecia universalis 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 affected people have a family history, the condition itself is not thought to be inherited.

Last updated: 5/17/2017

Diagnosis Diagnosis

A diagnosis of alopecia universalis 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: 5/17/2017

Treatment Treatment

No therapy is completely effective for alopecia universalis (AU) and management can be challenging. Although multiple treatments have been explored, no therapy is currently FDA-approved. Some promising therapies include:^[6]

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

In some people with AU, hair regrowth occurs without treatment, sometimes after many years.^[7]

There are measures that can be taken to minimize the effects of excessive sun exposure or discomfort. These may include:^[2]

Using sunscreen on the scalp, face, and all exposed areas
Wearing eyeglasses or sunglasses to protect the eyes from excessive 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

Last updated: 5/17/2017

Prognosis Prognosis

The course of alopecia universalis is highly unpredictable, and this uncertainty is one of the most difficult and frustrating aspects of the disease. Affected people 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: 5/17/2017

Statistics Statistics

We are unaware of prevalence statistics for alopecia universalis (AU) specifically. However, the prevalence of alopecia areata (of which AU is a subtype) is estimated to be 0.1% to 0.2% of all people. This would mean that approximately 318,000 to 636,000 people in the United States have some type of alopecia areata. Alopecia totalis or alopecia universalis are reported to occur at some point in 7% of people with alopecia areata.^[1]

Last updated: 5/17/2017

Do you have updated information on this condition? Let us know.

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 studying or have studied 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

Alopecia Areata Registry
MD Anderson Department of Dermatology
1400 Pressler Street – Unit 1452
Houston, Texas 77030
Toll-free: 1-866-837-1050
Fax: 713-794-1491
E-mail: alopeciaregistry@mdanderson.org
Web site: http://www.mdanderson.org/departments/alopecia/
Study coordinator: Joyce S. Osei, MPH, MHA
E-mail: jsosei@mdanderson.org

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: inquire@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: http://childrensalopeciaproject.org/contact/
National Alopecia Areata Foundation (NAAF)
65 Mitchell Boulevard
Suite 200-B
San Rafael, CA 94903
Telephone: 415-472-3780
Fax: 415-472-5343
E-mail: info@naaf.org
Website: http://www.naaf.org

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
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? Send us your suggestions.

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

Don’t Miss the NCATS Toolkit for Patient-Focused Therapy Development Event
August 31, 2017
Funding Opportunities Now Available for the Undiagnosed Diseases Network (UDN) Phase II
August 30, 2017
IRDiRC Goals 2017-2027: New Rare Disease Research Goals for the Next Decade
August 10, 2017

Related Diseases Related Diseases

The following diseases are related to Alopecia universalis. If you have a question about any of these diseases, you can contact GARD.

Alopecia areata

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. Submit a new question

What is the cause of my condition? And how many people get this? Can it be causing other side effects? See answer
Is this form of alopecia be reversible? Can the hair just grow back one day? See answer
My fiance has alopecia universalis. He has no family history of this condition. Recently, I read that the disease is hereditary. This worries me. Please help me understand our future children's chance of developing alopecia universalis. See answer
Is there a cure for alopecia universalis? See answer
I was born with alopecia universalis. I have recently had regrowth of thin sparse hairs. What can I expect from this condition? Will my hair ever grow back fully and can the condition be cured? See answer

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.
Treatments for Alopecia Areata. National Alopecia Areata Foundation. http://www.naaf.org/site/PageServer?pagename=about_alopecia_treatment. Accessed 5/17/2017.
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.