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Disease Profile

Frontal fibrosing alopecia

Prevalence
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.

Unknown

US Estimated

Europe Estimated

Age of onset

Adult

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ICD-10

L66.1

Inheritance

Autosomal dominant A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease.

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Autosomal recessive Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype.

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X-linked
dominant X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.

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X-linked
recessive Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder.

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Mitochondrial or multigenic Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.

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Multigenic or multifactor Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.

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Not applicable

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Other names (AKA)

FFA

Categories

Skin Diseases

Summary

Frontal fibrosing alopecia (FFA) is a form of lichen planopilaris that is characterized primarily by slowly progressive hair loss (alopecia) and scarring on the scalp near the forehead. In some cases, the eyebrows, eye lashes and/or other parts of the body may be involved, as well. Although it has been suggested that FFA may be due to hormonal changes or an autoimmune response, the exact cause of this condition is not yet known.[1][2][3] There is currently no cure for FFA; however, treatment with certain types of medications may stop or slow hair loss in some cases.[2]

Symptoms

Frontal fibrosing alopecia (FFA) is characterized primarily by hair loss (alopecia) and scarring on the scalp near the forehead. The band of hair loss on the front and sides of the scalp is usually symmetrical and slowly progressive (worsening over time). The skin in the affected area often looks normal but may be pale, shiny or mildly scarred.[2]

Approximately half of all affected people experience loss of eyebrows, as well. Less commonly, the eyelashes may also be involved. Some people with FFA develop hair loss in areas other than the scalp and face.[1][4]

In some cases, women with FFA also have female pattern hair loss, which is associated with thinning of hair on the scalp due to increased hair shedding and/or a reduction in hair volume.[2]

Cause

The exact underlying cause of frontal fibrosing alopecia (FFA) is unknown. FFA is thought to be an autoimmune condition in which an affected person's immune system mistakenly attacks the hair follicles (structures in the skin that make hair). Scientists also suspect that there may be a hormonal component since the condition most commonly affects post-menopausal women over age 50.[1][2]

Diagnosis

Frontal fibrosing alopecia is often suspected based on the presence of characteristic signs and symptoms. The diagnosis can be confirmed by examining a small sample of skin (skin biopsy) from the affected area. In some cases, laboratory studies may be ordered to rule out other conditions that cause similar features.[2][3]

Treatment

Unfortunately, there is currently no cure for frontal fibrosing alopecia (FFA).[2] Because the hair loss associated with this condition is thought to be caused by inflammation of hair follicles, treatment often involves using anti-inflammatory medications or ointments, such as corticosteroids, tetracyclines, or hydroxychloroquine (brand name Plaquenil), to reduce inflammation and suppress the body's immune system.[7] Medications that block the production of the male hormone 5-alpha reductase (finasteride) have been reported to stop further hair loss in some women.[2][4] Intralesional injection of medication and systemic therapies (taken by mouth) seem to be more effective than those applied to the skin. In many cases, a combination of treatments works best.[4] Some researchers continue to question whether treatment is effective or if hair loss in FFA just stops naturally.[8]

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

    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

    • The Cicatricial Alopecia Research Foundation has an information page on Frontal fibrosing alopecia. Click on the link to view this information page.
    • DermNet NZ is an online resource about skin diseases developed by the New Zealand Dermatological Society Incorporated. DermNet NZ provides information about this condition.

      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.
      • 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 Frontal fibrosing alopecia. Click on the link to view a sample search on this topic.

        Selected Full-Text Journal Articles

          References

          1. Arnold S. & Cooper S. Frontal fibrosing alopecia. Orphanet. May 2011; https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=254492.
          2. Singh D, Oakley A. Frontal fibrosing alopecia. DermNet NZ. January 2015; https://dermnetnz.org/hair-nails-sweat/frontal-fibrosing-alopecia.html.
          3. Basil M Hantash, MD. Scarring Alopecia. Medscape. February 26, 2016; https://emedicine.medscape.com/article/1073559-overview.
          4. Shapiro J & Otberg N. Lichen planopilaris. UpToDate. 2016; https://www.uptodate.com/contents/lichen-planopilaris.
          5. Tziotzios C, Stefanato CM, Fenton DA, Simpson MA, McGrath JA. Frontal fibrosing alopecia: reflections and hypotheses on aetiology and pathogenesis. Exp Dermatol. 2016 Nov; 25(11):847-852. https://www.ncbi.nlm.nih.gov/pubmed/27198858.
          6. Navarro-Belmonte MR, Navarro-López V, Ramírez-Boscà A, Martínez-Andrés MA, Molina-Gil C, González-Nebreda M, Asín-Llorca M. Case series of familial frontal fibrosing alopecia and a review of the literature. J Cosmet Dermatol. 2015 Mar; 14(1):64-9. https://www.ncbi.nlm.nih.gov/pubmed/25614294.
          7. Frequently Asked Questions. Cicatricial Alopecia Research Foundation. December 15, 2015; https://www.carfintl.org/faq.php.
          8. Tan KT, Messenger AG. Frontal fibrosing alopecia: clinical presentations and prognosis. British Journal of Dermatology. 2009; 160:75-79. https://www.ncbi.nlm.nih.gov/pubmed/18811690.

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