British Association of Dermatologists’ guidelines for the management of alopecia areata 2012

Abstract

The guidelines have been revised and updated in accordance with a predetermined scope, based on that used in the 2003 guidelines. Recommendations in these guidelines supersede those in the 2003 guidelines. The objectives of the guidelines are to provide up-to-date recommendations for the management of alopecia areata in adults and children and a summary of the evidence base. This guidance has been written by dermatologists and a patient representative. The draft guideline was made available for consultation and review by the British Association of Dermatologists’ (BAD) membership, the Primary Care Dermatological Society (PCDS), the British Dermatological Nursing Group (BDNG) and the board of Alopecia UK, a patient support organization. The final document was peer-reviewed by the Clinical Standards Unit of the BAD (made up of the Therapy and Guidelines subcommittee) prior to publication. These guidelines have been developed using the BAD’s recommendations1 and also with reference to the AGREE (Appraisal of Guidelines Research and Evaluation) instrument.2 PubMed, MEDLINE and EMBASE databases were searched from January 2002 to January 2012 and full relevant papers in the English language obtained. Additional, targeted searches were also carried out across these three databases, as well as a search on the Allied and Complementary Medicine Database (AMED); details of the search strategy are available as an Appendix online (see Supporting Information). The recommendations made are those that are currently considered best practice. Where possible they are based on randomized controlled trials (RCTs). However, in view of the limited evidence from RCTs, guidance is also based on less rigorously controlled studies, uncontrolled studies, on clinical experience, and on patient experience. These recommendations will be modified at intervals in light of new evidence. This document has been prepared on behalf of the BAD and is based on the best data available when the document was prepared. It is recognized that under certain conditions it may be necessary to deviate from the guidelines, and that the results of future studies may require some of the recommendations herein to be changed. Failure to adhere to these guidelines should not necessarily be considered negligent, nor should adherence to these recommendations constitute a defence against a claim of negligence. The proposed revision date for this set of recommendations is scheduled for 2017; where necessary, important interim changes will be updated on the BAD website. Alopecia areata is a chronic inflammatory disease that affects the hair follicle and sometimes the nail. The onset may be at any age and there is no known race or sex preponderance. Alopecia areata usually presents as patches of hair loss on the scalp but any hair-bearing skin can be involved. The affected skin may be slightly reddened but otherwise appears normal. Short broken hairs (exclamation mark hairs) are frequently seen around the margins of expanding patches of alopecia areata. The nails are involved in about 10% of patients referred for specialist advice. Hair follicles are preserved in alopecia areata and the potential for recovery of hair growth is maintained, even in longstanding disease. One study from Japan reported that spontaneous remission within 1 year occurred in 80% of patients with a small number of circumscribed patches of hair loss.3 Data from secondary and tertiary referral centres are less favourable indicating that 34–50% of patients will recover within 1 year. Almost all will experience more than one episode of the disease, and 14–25% progress to total loss of scalp hair (alopecia totalis, AT) or loss of the entire scalp and body hair (alopecia universalis, AU), from which full recovery is unusual (< 10%).4, 5 Disease severity at presentation is the strongest predictor of long-term outcome. In an Italian study, 191 patients with alopecia areata who presented to a university dermatology clinic between 1983 and 1990 were contacted by telephone in 2005 to give self-reports of their clinical status.6 Patients with less severe disease at presentation were more likely to report being free of disease at follow-up (68% with less than 25% hair loss initially; 32% with 25–50% hair loss initially; 8% with more than 50% hair loss initially). Patients with more severe disease initially were also more likely to report worsening patterns of alopecia such as AT and AU. The prognosis is also less favourable when onset occurs during childhood4, 7-9 and in ophiasis.9 The concurrence of atopic disease has been reported to be associated with a poor prognosis3, 9 but this has been disputed.10 About 20% of people with alopecia areata have a family history of the disease indicating a genetic predisposition.11 Associations have been reported with a variety of genes, including major histocompatibility complex (MHC) and cytokine genes, suggesting that the genetic predisposition is multifactorial in nature. A genome-wide association study confirmed the link with the MHC genes and also identified associations with other genes involved in regulating immune and inflammatory responses, and with some genes expressed in the hair follicle.12 The hair follicle lesion is probably mediated by T lymphocytes.13 The association between alopecia areata and other autoimmune diseases suggests that alopecia areata is itself an autoimmune disease although this is unproven. It has been proposed that the hair follicle is an immunologically ‘privileged tissue’ which is sheltered from immune surveillance by autoreactive T cells, and that failure of such immune privilege plays a key role in the pathogenesis of alopecia areata.14, 15 The diagnosis of alopecia areata is usually straightforward although the following may cause diagnostic difficulties: Trichotillomania – this condition probably causes most confusion and it is possible that it coexists with alopecia areata in some cases. The incomplete nature of the hair loss in trichotillomania and the fact that the broken hairs are firmly anchored in the scalp (i.e. they remain in the growing phase, anagen, unlike exclamation mark hairs) are distinguishing features. Tinea capitis – the scalp is inflamed in tinea capitis but the signs may be subtle. Early scarring alopecia. Telogen effluvium. Anagen effluvium (drug-induced) may mimic diffuse alopecia areata. Systemic lupus erythematosus. Secondary syphilis. Dermoscopy can aid the diagnosis of alopecia areata. Regular round yellow dots are commonly seen in areas of hair loss and can indicate active disease progression. Dermoscopy also highlights common features seen in this condition such as dystrophic hairs with fractured tips (exclamation mark hairs) and hairs fractured before emergence from the scalp (cadaverized hairs). These findings are not present in triangular alopecia, trichotillomania or localized scarring conditions, which are sometimes considered within the differential of alopecia areata.16 Occasionally, alopecia areata presents as diffuse hair loss which can be difficult to diagnose. The clinical course often reveals the true diagnosis but a biopsy may be necessary in some cases. Investigations are unnecessary in most cases of alopecia areata. When the diagnosis is in doubt appropriate tests may include fungal culture, skin biopsy, serology for lupus erythematosus or serology for syphilis. The increased frequency of autoimmune disease in patients with alopecia areata is probably insufficient to justify routine screening. One small case series suggested that iron deficiency is more common in women with alopecia areata than the population at large17 but this was not confirmed in two subsequent studies,18, 19 and routine testing for iron status is not recommended. There are no published studies demonstrating a treatment response to iron replacement therapy. An overriding consideration in the management of alopecia areata is that, although the disease may have a serious psychological effect, it has no direct impact on general health that justifies the use of hazardous treatments, particularly of unproven efficacy. In addition, many patients, although by no means all, experience spontaneous regrowth of hair. However, the psychological effects of alopecia may impact on general health and depends on the individual’s coping strategy when dealing with an altered body image, which can result in higher levels of anxiety and a greater risk of depression leading to social, work-related and personal problems.20 An explanation of alopecia areata, including discussion of the nature and course of the disease and the available treatments, is essential. Some patients are profoundly upset by their alopecia and may require psychological support. Many find it difficult to disclose their alopecia to family members and friends and struggle to find the answers to their medical and many practical questions. Contact with other patient experts and patient support groups can help individuals cope with the changing aspects of alopecia and provide support to find a new level of self-acceptance of their altered body image. Alopecia areata in children can be particularly difficult. If a parent feels there is a significant change in a child’s needs (withdrawn, low self-esteem, failing to achieve at school, change in behaviour), referral to a paediatric clinical psychologist, educational psychologist or social worker may be needed. It is important to consider both the positive and negative aspects of active treatment in this chronic condition. Some patients do respond well to treatment. However, treatment can be uncomfortable for the patient, time-consuming and can be associated with undesirable side-effects. It may also alter the patient’s attitude to their hair loss. Some patients find it difficult to cope with relapse following or during initially successful treatment and they should be forewarned of this possibility. These considerations are particularly important in children where the social disruption and focusing of the child’s attention on their hair loss, which may result from active treatment, have to be carefully weighed against the potential benefits. On the other hand, some patients are appreciative that something has been tried, even if it does not work. An individual’s reaction to alopecia will vary depending on their own perceptions of body image, self-esteem, coping strategies, personality traits and their social support network. Commonly, people may feel self-conscious, conspicuous, angry, rejected, embarrassed or different and they may behave in a shy, cautious, aggressive, retreating, evasive or defensive (SCARED) manner.21 It is important to mention self-acceptance particularly in those with long-standing, extensive and persistent alopecia areata. A number of treatments can induce hair growth in alopecia areata but none has been shown to alter the long-term course of the disease. The high rate of spontaneous remission makes it difficult to assess efficacy, particularly in mild forms of the disease. Some trials have been limited to patients with severe alopecia areata where spontaneous remission is unlikely. However, these patients tend to be resistant to all forms of treatment and the failure of a treatment in this setting does not exclude efficacy in mild alopecia areata. There are numerous case reports and uncontrolled case series claiming response of alopecia areata to diverse treatments. However, few treatments have been subjected to RCTs for there are few published data on long-term A review of RCTs in alopecia areata that one evidence of and none long-term However, the review not consider or treatment to the of RCTs for these alopecia areata is a for many remission occurs in up to 80% of patients with limited hair loss of (< 1 patients may be by with that regrowth be within of the of any The prognosis in longstanding extensive alopecia is poor and a may be a in such patients than in treatments that are to be in this the of levels of evidence Appendix of evidence are used to alopecia areata but the evidence for their is In a of in patients with alopecia areata, more patients with the at with but the result to In a of patients with to severe alopecia areata were to treatment to one of the scalp and to the other of treatment, more with at 50% regrowth of hair of one of under an may be in some In a study of patients who for a of under an on out of for in long-term hair regrowth in patients The study initially patients use the treatment on one of the and no hair regrowth occurred on the is a common of treatment with of evidence hair regrowth at the of in some and reported that of hair in out of with in patients with alopecia areata, and in of with in The about 9 In a study from of patients full regrowth with of the response being in those with than patches of in This is most for hair loss of limited and for such as the and are commonly is the in the An of will a of hair growth about in may be the being patient may also be by a UK, The should be between and reported that of patients regrowth of hair at three of using the with one of 15 with The results were less favourable in alopecia than in localized alopecia. at the of is a of particularly if is but this usually a few at the or the use of higher of should be as this may cause skin There is a risk of and if are used to the for There are two case reports of in patients for treatment of alopecia are not appropriate in alopecia or in extensive disease. Systemic of evidence treatment with will regrowth of hair in some One small controlled study reported that of patients with a course of at more than 25% hair in most patients, treatment is to hair growth and the response is usually insufficient to justify the There are published case series of treatment different and for 5 The in treatment and patient it difficult to these studies about of patients with extensive alopecia to a response to than 10% of those with disease and In the controlled patients were with or for Patients hair regrowth at but this was not There is published on long-term In a small case series of children with severe alopecia areata who were with the long-term follow-up was poor a or response in children at 1 have not been reported with of in alopecia areata. However, and long-term of are well known and and in view of these it is not possible to support their use there is evidence of efficacy. Contact was by and in The that have been used in the treatment of alopecia areata and from when it was to be against in the nor are One is and should be for by the is more in and is usually the of The for using was by The patient is using a of to a small of the the scalp is with a of at and this is at The is increased at treatment a mild reaction is obtained. Some one of the scalp initially to between a treatment response and spontaneous recovery if hair regrowth hair regrowth is both of the scalp are In patients with severe longstanding alopecia, where spontaneous recovery is this is are on patients should be to a response is most the frequency of treatment. In patients where full regrowth of hair is treatment can be will usually respond to although this be A review of all the published studies of that of patients achieve a response but the of response was Patients with extensive hair loss are less likely to reported features include the of onset and a positive family In most studies, treatment has been if no response is obtained. In a case series from significant regrowth occurred in about of patients of treatment but this increased to of treatment, suggesting that more treatment is The response in patients with was less favourable at and this was not by treatment 9 may following or during treatment. In the relapse following successful treatment occurred in of case report series of in children with alopecia areata reported response of and A study a response in children with severe alopecia areata but 10% effects patients will during This is usually but may the treatment is the most common but the risk can be by of the effects include which may be and both and may if is used in patients with patients should be of this risk before on treatment. Contact has been in use for and no long-term have been Contact is an treatment that a Patients should be about the nature of the be an and give be to with the by including medical and and other members of the patient’s the should and There are no data on the of during and it should not be used in women nor in women to is by should be in the and patients should a or for following There are uncontrolled studies of A treatment for alopecia areata, using all of or or body claiming of up to have reported low response or suggested that the response was no than the course of the although these were also The relapse rate following treatment is high and treatment is usually to hair which may to an high An study reported a greater frequency of hair regrowth in alopecia areata in patients with with controlled trials in patients with extensive alopecia areata using or to these of these studies reported a treatment response during an but uncontrolled of the In one study and in extensive alopecia areata, regrowth of hair occurred more frequently in those but few a is in There are a small number of case report series of or other in the treatment of alopecia The of makes the response difficult to but a small of patients to achieve In one study, of patients with extensive alopecia areata hair The published data indicate that needs to be frequently and in a high to a reaction in to be of hair use in The of as an and as a it a in alopecia areata and this is by there are a small number of published uncontrolled trials with low patient the evidence that does hair regrowth in some patients with alopecia areata is However, as has to be is not active are a major consideration in patients with severe alopecia areata, the response rate is probably low to justify the response to treatment was seen in a case series of patients with to severe alopecia areata with for is a of treatment of with the and In a study of patients with or regrowth of when with for with no regrowth in a However, a controlled study in patients with alopecia no significant response to or and regrowth was seen in a patient in a controlled study of in A more is to these The response of alopecia areata to has to date that are There are reports of alopecia areata in patients for other and in an study in patients with to severe alopecia areata there was no response to treatment with In a in patients with chronic severe alopecia areata, there was no significant response to an with evidence of efficacy, from uncontrolled case series or from controlled or controlled for a number of treatments. of evidence uncontrolled case series have response to In an uncontrolled study of patients with severe alopecia areata hair of the treatment, recovery and a regrowth of hair. or relapse occurred in of the 15 of evidence In a review of patients with with with or regrowth of including three of patients with of evidence is an that has and Early uncontrolled studies of use in alopecia areata reported and negative A more in patients with alopecia areata reported remission at in 50% of patients with none in the of evidence An was used to alopecia areata in or regrowth was seen in of no growth occurred in patches In patches of alopecia areata were for with a was seen in results response were in a study of treatment in children with alopecia areata. to of evidence In a 19 out of patients a treatment response at with out of patients in the of evidence In a patients with severe alopecia by with patients, the a significant in for depression and but there was no between groups in of hair the negative on hair growth this study highlights the role of treatments in patients with alopecia areata. with the impact of alopecia areata depends on the individual’s to with an altered body and their perceptions of When individuals often have an of being particularly when about hair from social as many do not feel at their and can be used as to cope with alopecia areata. However, to a hair can be an experience, to the variety of different and to can be limited depending on an individual’s as in from to are the most are to give the of hair growing from the they are and in a variety of and However, all such as and if will to the of the in condition and the of hair. hair depends on where the hair has been and the of the if the is or made to hair and will if in in give some the to in and However, they are and consideration is when a particularly when online or The on for a for hair is available to patients who are to or who have a skin condition made by individuals with alopecia areata or hair loss, there are to a of hair or use a that is hair or with find it particularly when and as a from has been as well as dealing with a altered can be or hair and can be can be to but with can and to can be and can help to to an has and can be up to or depending on the can be that are and can on for up to in from for and have to the of The from to with a at and but can up to Some in the skin but to on of and is in Appendix 1 should include the age of history of family history and other the severity and of alopecia or and the of changes should be of was the of the consultation – was treatment and to the treatments, including and – was The details of evidence are Alopecia areata is difficult to and few treatments have been in The to spontaneous remission and the of effects on general health are important considerations in and not is the best in many cases. On the other hand, alopecia areata may cause psychological and social and in some particularly those seen in secondary it may be a chronic and persistent disease extensive or hair loss. In those cases where treatment is appropriate there is evidence to support the following of recommendations are in Appendix with probably regrowth of hair in some patients with mild to disease but there are no data on long-term hair regrowth at the of The is a few and it is the long-term is Contact is the treatment in severe alopecia areata but it is not to and hair regrowth in 50% of It is the treatment likely to be in although the response rate is It may cause but serious are and are by dermatologists for limited alopecia areata, and are but there is no evidence that they are or and have also been used to alopecia areata. However, in view of the serious and evidence of efficacy, none can be at this may be in a to However, are often and many are to use treatments such as in on patient support can be in the BAD on alopecia areata are to Clinical Standards for out the searches and in the The can provide a limited to on in and to free is in available In and are to hair are available to patients who are to or who have a skin condition made by Appendix search The is not for the or of any by the than should be to the for the