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

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 of spontaneous remission it difficult to particularly in of the disease. Some trials have been limited to patients with severe alopecia areata where spontaneous remission is However, these patients to be to all of treatment and the failure of a treatment in this does not in alopecia areata. 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However, in view of the serious and evidence of 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 with a level of evidence should not be used as a for a randomized controlled randomized controlled for and Clinical 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 of of the has a or in any of the treatments as a to who and for the treatment of hair loss This is an updated guideline prepared for the British Association of Dermatologists’ (BAD) Clinical Standards made up of the Therapy Guidelines of the Clinical Standards Unit Dermatological Nursing Clinical Standards Guidelines in 2003 by the British Association of and updated Appendix search