Changes

September 2024 — minor update. Ritlecitinib added to the specialist management options for severe alopecia areata. 

Update

Goals

To support primary healthcare professionals to:

• Make an accurate assessment and diagnosis of alopecia areata and exclude other conditions that present similarly.
• Manage appropriately in primary care.
• Arrange referral to dermatology if appropriate.
• Provide information and advice about the condition.

Outcome measures

Audit criteria

QOF indicators

QIPP - Options for local implementation

NICE quality standards

What is it?

• Alopecia areata is a chronic, inflammatory condition affecting the hair follicles which leads to sudden onset of non-scarring alopecia (hair loss where the hair follicles are generally preserved).
  • Any hair-bearing skin can be involved, but it most commonly affects the scalp or beard and, less frequently, the eyebrows and eyelashes.
    • Hair loss is usually patchy, producing circular or oval areas of loss. Diffuse hair loss is more unusual.
    • Total loss of scalp hair (alopecia totalis) or scalp and body hair (alopecia universalis) is uncommon.
    • There may be a band-like pattern of hair loss at the occipital and temporal scalp margin (ophiasis pattern) or hair loss at the vertex with occipital and temporal sparing (sisaipho or ophiasis inversus pattern, mimicking male androgenetic hair loss).
    • Occasionally, there may be an 'overnight greying' or 'white overnight' effect due to loss of pigmented hairs with sparing of white hairs.
  • Nail changes are seen in 10–15% of people with alopecia areata, typically those with more severe disease.
  • Images of the different types of alopecia areata and associated nail changes may be seen on the DermNet website, or the website of the Primary Care Dermatology Society (PCDS). 

[Messenger, 2012]  [Strazzulla, 2018a; Sterkens, 2021; DermNet, 2022; BMJ Best Practice, 2023]

What causes it?

Alopecia areata is thought to be an autoimmune disease, and occurs when hairs are prematurely converted from the growth (anagen) to the loss (telogen) phase, resulting in dystrophic, miniaturised hairs [Strazzulla, 2018a; Sterkens, 2021; DermNet, 2022].

• The cause of alopecia areata is unknown but multiple factors are likely to be involved including [Fukumoto, 2021; Simakou, 2019]:
  • Genetics — about 20% of people with alopecia areata have a positive family history [Messenger, 2012]. Observed heritability in first-degree relatives and twin studies further supports a genetic basis for the condition [Hordinsky, 2015; Spano, 2015a]. 
  • Autoimmune reactions — defects occur in localized immunosuppressive mechanisms which are thought to be T-cell mediated. The anagen hair follicle is usually a site of 'immune privilege' where mechanisms prevent autoimmune attack. It is theorized that in alopecia areata this is disrupted due to T-cell interaction with aberrant antigens expressed by hair follicle keratinocytes. There is an association with other autoimmune conditions, including systemic lupus erythematosus, thyroiditis, vitiligo, pernicious anaemia and atopy [Hordinsky, 2015; Spano, 2015a; Sterkens, 2021; Lepe, 2022; BMJ Best Practice, 2023].
  • Stress — stressful life events may be related to the onset and/or progression of alopecia areata [Hordinsky, 2014; Fricke, 2015; Azzawi, 2018]. 
  • Lifestyle factors — smoking appears to increase the risk of alopecia areata, and there is speculation but no strong evidence that other lifestyle factors such as alcohol, obesity, diet and sleep disturbance may be involved in pathogenesis [Minokawa, 2022].
  • Neurogenic — changes in the peripheral nervous system may alter the release of neuropeptides and influence inflammatory reactions [Hordinsky, 2015; Kim, 2022].

How common is it?

• Alopecia areata is a relatively common condition with an estimated prevalence of 1 in 1000 people and a lifetime incidence of approximately 2% [Lee, 2020; Sterkens, 2021; DermNet, 2022; BMJ Best Practice, 2023].
• A UK population-based cohort study of 4.16 million adults and children published in 2022 using primary care records between 2009 and 2018 found [Harries, 2022]:
  • Point prevalence in 2018 was 0.58% in adults.
  • Incidence rate was 0.26 per 1000 person-years. 6765 people developed alopecia areata for the first time over the study period.
  • Onset peaked between 25–29 years of age. 
  • People of non-white ethnicity were more likely to present with alopecia areata, particularly those of Asian ethnicity.
• A US retrospective study (1990–2009) estimated the incidence of alopecia areata was 20.9 per 100,000 person-years, with a cumulative lifetime incidence of 2.1% [Mirzoyev, 2014].
• Alopecia areata can present at any age, and males and females are affected equally [Harries, 2010; Messenger, 2012; Hordinsky, 2015; Xu, 2017].
  • 1–2% of people present before the age of 2 years [Xu, 2017].
  • Peak incidence occurs in the second and third decades of life, and in most onset occurs before the fourth decade [DermNet, 2022].

What are the complications?

• Possible complications of alopecia areata include:
  • Psychosocial impact — altered body image, reduced self-esteem, social withdrawal, increased risk of anxiety, depression, adjustment disorder and possibly an increased risk of suicide.
  • Behaviour change — particularly in children, including school refusal and reduced academic performance.
  • Reduced quality of life.

[Messenger, 2012; Fricke, 2015; Xu, 2017; Sterkens, 2021; Toussi, 2021]

What is the prognosis?

The prognosis of alopecia areata is unpredictable and variable in different studies. Disease extent and pattern at presentation are predictors of long-term outcome [Harries, 2010; Messenger, 2012].

• There tends to be unpredictable episodes of relapsing and remitting disease [BMJ Best Practice, 2023]. Note that the literature varies considerably in reports of remission and progression rates, although there is more consensus about prognostic indicators.
• Spontaneous remission within one year may occur, with studies suggesting this occurs in between 34 to 50% of people [Harries, 2010; Messenger, 2012]. Hair regrowth is more likely in those with limited patches of hair loss of less than one year duration, but most will later experience further episodes [Messenger, 2012; Rossi, 2019]. The British Association of Dermatologists (BAD) guidelines quote a 1965 paper from Japan where spontaneous remission within a year was reported in 80% of patients with small numbers of circumscribed patches of hair loss, but also notes that data from referral centres indicates less favourable spontaneous remission rates of 34 to 50%, which is in line with figures quoted in the more recent expert reviews [Ikeda, 1965; Messenger, 2012; Rossi, 2019; Lepe, 2022].
• It has been estimated that 15 to 25% of people with patchy disease will progress to develop alopecia totalis or alopecia universalis [Novoa-Candia, 2020; Lepe, 2022].
  • Fewer than 10% of people with extensive disease have a sustained remission [Harries, 2010; Sterkens, 2021].
• A person may experience several episodes of hair loss and regrowth during their lifetime. A poorer prognosis may be associated with [Harries, 2010; Messenger, 2012; Xu, 2017; DermNet, 2022]:
  • Onset in childhood.
  • Family history of alopecia areata.
  • Longstanding, extensive alopecia, including alopecia totalis or universalis.
  • Involvement of the scalp margin (ophiasis) and/or nails changes.
  • A history of atopy or other autoimmune diseases such as systemic lupus erythematosus, thyroiditis, pernicious anaemia or vitiligo. 

When should I suspect alopecia areata?

Suspect a diagnosis of alopecia areata if there are typical clinical features, and if other causes of hair loss have been excluded.

• Hair loss typically affects any hair-bearing area (most commonly the scalp or within facial hair such as the beard) and is usually patchy and of sudden onset.
  • Patches of hair loss are usually round, well-circumscribed and smooth. Patches may coalesce into larger areas of alopecia. More rarely, hair loss may be diffuse.
  • The skin is normal-coloured or slightly red without scarring (the follicular openings are still present).
  • Exclamation mark hairs (short broken hairs which taper proximally) may be seen around the margin, or in any part of the patch, during active disease.
• Hair loss is usually asymptomatic, although occasionally itch, tingling, burning, or pain may occur prior to hair loss or with disease activity. Where symptoms such as these are present, other causes such as tinea capitis should be considered and excluded.
• Nail changes may occur, and include pitting, onycholysis (loosening), splitting, longitudinal ridging, red lanula, koilonychia (concave outer surface), and leukonychia (white patches under the nails).

How should I assess a person with suspected alopecia areata?

If a diagnosis of alopecia areata is suspected:

• Ask the person about:
  • How the hair loss is affecting them and the impact on their self-confidence, self-esteem, and quality of life.
  • Any known trigger such as emotional stress, and any associated anxiety and/or depression. If identified, these should be managed appropriately. See the CKS topics on Post-traumatic stress disorder, Generalized anxiety disorder, Depression in children, and Depression for more information.
  • Their diet and nutrition.
  • Their coping strategies and social support network.
  • Any previous episodes of hair loss.
  • Any current or past treatments (including over-the-counter preparations), and their effectiveness.
  • Any family history of hair loss.
  • Any history or family history of associated atopy or autoimmune disease, such as systemic lupus erythematosus, thyroid disease, pernicious anaemia or vitiligo.
• Examine the person:
  • Determine the nature and extent of hair loss on the scalp (patchy, diffuse, total, ophiasis or sisaipho pattern) and other sites, including eyelashes, eyebrows, axillary, and pubic hair (if appropriate). 
  • Severity at onset may impact management choices and prognosis (poorer outcome with more extensive disease), so it is helpful to assess this. Alopecia areata is considered severe when more than 50% of the scalp surface is involved, mild when it is patchy and involves less than 25% of the scalp, and moderate between these two categories.
  • Check for signs of skin inflammation, erythema, scaling, or scarring, which may suggest an alternative diagnosis.
  • Assess for evidence of hair regrowth (short, fine, tapered hair or depigmented hair), which affects options for management.
  • Assess for any associated nail changes.
• Consider performing the 'pull test' to identify if there is active hair shedding.
  • This involves grasping a small section of hairs at the periphery of a hair loss patch between the finger and thumb (about 50–60 hairs) and tugging gently but firmly (it should not be painful), sliding the fingers along the hair shaft.
  • The test is positive (confirming active shedding) if more than three hairs (or greater than 10% of hairs) are pulled away from the scalp.
  • Note: the test shows high inter-observer variation, and the definition of how many hairs constitutes a positive test varies in the literature. 
• Routine investigations are not usually needed, but consider the following tests if there is diagnostic uncertainty or atypical features:
  • Full blood count, ferritin, and thyroid function tests, particularly if telogen effluvium is suspected. See the CKS topics on Female pattern hair loss (female androgenetic alopecia) and Male pattern hair loss (male androgenetic alopecia) for more information.
  • Skin scrapings and hair samples for fungal culture and sensitivity. See the CKS topic on Fungal skin infection - scalp for more information.
  • In secondary care, or where there is expertise in primary care, trichoscopy (use of a dermatoscope) and skin biopsy may also be helpful where there is diagnostic doubt.
    • Typical features on trichoscopy include the presence of yellow dots, black dots (dystrophic and damaged telogen hairs), broken hairs or the classic exclamation mark hairs at the borders of the affected areas.
    • Following skin biopsy, histopathology may show a 'bee-swarm pattern' of dense lymphocytic infiltrate around anagen hair follicles, and a decrease in the ratio of anagen to telogen follicles.
• Routine testing for associated autoimmune conditions is not recommended.

What else might it be?

Alternative conditions that may present similarly to alopecia areata include:

• Patchy hair loss
  • Tinea capitis — see the CKS topic on Fungal skin infection - scalp for more information.
  • Trichotillomania — a psychiatric condition where people pull their own hair out. Hair loss is typically incomplete, asymmetrical, and has an unusual shape. There are usually multiple broken hairs of varying length. Single or multiple areas may be affected, including eyebrows and eyelashes. See the CKS topic on Obsessive-compulsive disorder for more information.
  • Traction alopecia — hair loss secondary to pulling on the roots, commonly caused by hair styling techniques (for example tight braids or pony tails), and usually involving the scalp margins causing temporal hair thinning.
  • Androgenetic alopecia — there is a typical pattern of hair loss over the crown where pigmented terminal hairs are progressively replaced by finer hairs. Both men and women can be affected. In women, hair loss may be more diffuse, with a reduction in hair density over the crown and frontal scalp, with the frontal hairline usually preserved. See the CKS topics on Female pattern hair loss (female androgenetic alopecia) and Male pattern hair loss (male androgenetic alopecia) for more information.
  • Scarring (cicatricial) alopecia — caused by inflammatory disorders that target and destroy the hair follicle, resulting in permanent alopecia, such as scleroderma, discoid lupus, lichen planus, or shingles. See the CKS topic on Shingles for more information.
  • Secondary syphilis — typically causes a moth-eaten, patchy hair loss. See the CKS topic on Syphilis for more information.
• Diffuse hair loss
  • Telogen effluvium — describes the loss of individual hairs earlier than normal because of a more rapid hair growth cycle, often associated with profound shedding or a rapid onset of hair loss.
    • Acute generalized telogen hair loss over the whole scalp can occur about 3 months after a significant event, such as physical or psychological stress. Bi-temporal recession is a common sign in women. Hair loss lasts for 3–6 months and then the hair regrows. Telogen gravidarum is telogen hair loss typically seen 2–3 months after childbirth.
    • Chronic diffuse telogen hair loss can occur as a result of stress, thyroid disorders, iron deficiency anaemia, and malnutrition, but often no cause is found.
  • Anagen effluvium — describes drug-induced hair loss, for example following chemotherapy, tricyclic antidepressants, allopurinol, beta-blockers, nitrofurantoin, or retinoids.

Scenario: Management of alopecia areata

From age 24 months onwards.

Search strategy

Scope of search

A literature search was conducted for guidelines, systematic reviews and randomized controlled trials on primary care management of alopecia areata.

Search dates

February 2018 - February 2023

Key search terms

Various combinations of searches were carried out. The terms listed below are the core search terms that were used for EBSCO Medline.

• (MH "Alopecia Areata")
• AB alopecia areata OR alopecia totalis OR alopecia universalis OR TI alopecia areata OR alopecia totalis OR alopecia universalis

Sources of guidelines

• National Institute for Health and Care Excellence (NICE)
• Scottish Intercollegiate Guidelines Network (SIGN)
• Royal College of Physicians
• Royal College of General Practitioners
• Royal College of Nursing
• NICE Evidence
• World Health Organization
• Guidelines International Network
• TRIP database
• Agency for Healthcare Research and Quality
• National Health and Medical Research Council (Australia)
• Royal Australian College of General Practitioners
• British Columbia Medical Association
• Canadian Medical Association
• Alberta Medical Association
• Michigan Quality Improvement Consortium
• Singapore Ministry of Health
• National Resource for Infection Control
• RefHELP NHS Lothian Referral Guidelines
• Medline (with guideline filter)
• Driver and Vehicle Licensing Agency
• NHS Health at Work (occupational health practice)

Sources of systematic reviews and meta-analyses

• The Cochrane Library:
  • Systematic reviews
  • Protocols
  • Database of Abstracts of Reviews of Effects
• Medline (with systematic review filter)
• EMBASE (with systematic review filter)

Sources of health technology assessments and economic appraisals

• NIHR Health Technology Assessment programme
• The Cochrane Library:
  • NHS Economic Evaluations
  • Health Technology Assessments
• Canadian Agency for Drugs and Technologies in Health
• International Network of Agencies for Health Technology Assessment

Sources of randomized controlled trials

• The Cochrane Library:
  • Central Register of Controlled Trials
• Medline (with randomized controlled trial filter)
• EMBASE (with randomized controlled trial filter)

Sources of evidence based reviews and evidence summaries

• Drug and Therapeutics Bulletin
• TRIP database
• Central Services Agency COMPASS Therapeutic Notes

Sources of national policy

• Department of Health
• Health Management Information Consortium (HMIC)

Patient experiences

• Healthtalk
• BMJ - Patient Journeys

Sources of medicines information

The following sources are used by CKS pharmacists and are not necessarily searched by CKS information specialists for all topics. Some of these resources are not freely available and require subscriptions to access content.

• British National Formulary (BNF)
• electronic Medicines Compendium (eMC)
• European Medicines Agency (EMEA)
• Medicines and Healthcare products Regulatory Agency (MHRA)
• REPROTOX
• Scottish Medicines Consortium
• Stockley's Drug Interactions
• TERIS
• TOXBASE
• Micromedex
• UK Medicines Information

Stakeholder engagement

Our policy

The external review process is an essential part of CKS topic development. Consultation with a wide range of stakeholders provides quality assurance of the topic in terms of:

• Clinical accuracy.
• Consistency with other providers of clinical knowledge for primary care.
• Accuracy of implementation of national guidance (in particular NICE guidelines).
• Usability.

Principles of the consultation process

• The process is inclusive and any individual may participate.
• To participate, an individual must declare whether they have any competing interests or not. If they do not declare whether or not they have competing interests, their comments will not be considered.
• Comments received after the deadline will be considered, but they may not be acted upon before the clinical topic is issued onto the website.
• Comments are accepted in any format that is convenient to the reviewer, although an electronic format is encouraged.
• External reviewers are not paid for commenting on the draft topics.
• Discussion with an individual or an organization about the CKS response to their comments is only undertaken in exceptional circumstances (at the discretion of the Clinical Editor or Editorial Steering Group).
• All reviewers are thanked and offered a letter acknowledging their contribution for the purposes of appraisal/revalidation.
• All reviewers are invited to be acknowledged on the website. All reviewers are given the opportunity to feedback about the external review process, enabling improvements to be made where appropriate.

Stakeholders

• Key stakeholders identified by the CKS team are invited to comment on draft CKS topics. Individuals and organizations can also register an interest to feedback on a specific topic, or topics in a particular clinical area, through the Getting involved section of the Clarity Informatics website.
• Stakeholders identified from the following groups are invited to review draft topics:
  • Experts in the topic area.
  • Professional organizations and societies (for example, Royal Colleges).
  • Patient organizations, Clarity has established close links with groups such as Age UK and the Alzheimer’s Society specifically for their input into new topic development, review of current topic content and advice on relevant areas of expert knowledge.
  • Guideline development groups where the topic is an implementation of a guideline.
  • The British National Formulary team.
  • The editorial team that develop MeReC Publications.
• Reviewers are provided with clear instructions about what to review, what comments are particularly helpful, how to submit comments, and declaring interests.

Patient engagement

Clarity Informatics has enlisted the support and involvement of patients and lay persons at all stages in the process of creating the content which include:

• Topic selection
• Scoping of topic
• Selection of clinical scenarios
• First draft internal review
• Second draft internal review
• External review
• Final draft and pre-publication

Our lay and patient involvement includes membership on the editorial steering group, contacting expert patient groups, organizations and individuals.

Evidence exclusion criteria

Our policy

Scoping a literature search, and reviewing the evidence for CKS is a methodical and systematic process that is carried out by the lead clinical author for each topic. Relevant evidence is gathered in order that the clinical author can make fully informed decisions and recommendations. It is important to note that some evidence may be excluded for a variety of reasons. These reasons may be applied across all CKS topics or may be specific to a given topic.

Studies identified during literature searches are reviewed to identify the most appropriate information to author a CKS topic, ensuring any recommendations are based on the best evidence. We use the principles of the GRADE and PICOT approaches to assess the quality of published research. We use the principles of AGREE II to assess the quality of published guidelines.

Standard exclusions for scoping literature:

• Animal studies
• Original research is not written in English

Possible exclusions for reviewed literature:

• Sample size too small or study underpowered
• Bias evident or promotional literature
• Population not relevant
• Intervention/treatment not relevant
• Outcomes not relevant
• Outcomes have no clear evidence of clinical effectiveness
• Setting not relevant
• Not relevant to UK
• Incorrect study type
• Review article
• Duplicate reference

Organizational, behavioural and financial barriers

Our policy

The CKS literature searches take into consideration the following concepts, which are discussed at the initial scoping of the topic.

• Feasibility
  • Studies are selected depending on whether the intervention under investigation is available in the NHS and can be practically and safely undertaken in primary care.
• Organizational and Financial Impact Analysis
• Studies are selected and evaluated on whether the intervention under investigations may have an impact on local clinical service provision or national impact on cost for the NHS. The principles of clinical budget impact analysis are adhered to, evaluated and recorded by the author. The following factors are considered when making this assessment and analysis.
  • Eligible population
  • Current interventions
  • Likely uptake of new intervention or recommendation
  • Cost of the current or new intervention mix
  • Impact on other costs
  • Condition-related costs
  • In-direct costs and service impacts
  • Time dependencies
• Cost-effectiveness or cost-benefit analysis studies are identified where available. 

We also evaluate and include evidence from NICE accredited sources which provide economic evaluations of recommendations, such as NICE guidelines. When a recommended action may not be possible because of resource constraints, this is explicitly indicated to healthcare professionals by the wording of the CKS recommendation.

Declarations of interest

Our policy

Clarity Informatics requests that all those involved in the writing and reviewing of topics, and those involved in the external review process to declare any competing interests. Signed copies are securely held by Clarity Informatics and are available on request with the permission of the individual. A copy of the declaration of interest form which participants are asked to complete annually is also available on request. A brief outline of the declarations of interest policy is described here and full details of the policy is available on the Clarity Informatics website. Declarations of interests of the authors are not routinely published, however competing interests of all those involved in the topic update or development are listed below. Competing interests include:

• Personal financial interests
• Personal family interest
• Personal non-financial interest
• Non-personal financial gain or benefit

Although particular attention is given to interests that could result in financial gains or losses for the individual, competing interests may also arise from academic competition or for political, personal, religious, and reputational reasons. An individual is not obliged to seek out knowledge of work done for, or on behalf of, the healthcare industry within the departments for which they are responsible if they would not normally expect to be informed.

Who should declare competing interests?

Any individual (or organization) involved in developing, reviewing, or commenting on clinical content, particularly the recommendations should declare competing interests. This includes the authoring team members, expert advisers, external reviewers of draft topics, individuals providing feedback on published topics, and Editorial Steering Group members. Declarations of interest are completed annually for authoring team and editorial steering group members, and are completed at the start of the topic update and development process for external stakeholders.

Competing interests declared for this topic:

None.