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Home , Beard, Scarring hair loss, Telogen effluvium

BMJ 2013;347:f6112 doi: 10.1136/bmj.f6112 (Published 31 October 2013) Page 1 of 3

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PICTURE QUIZ

Sudden onset loss and colour change

R C Lamb specialty trainee year 6 registrar, M Porter consultant dermatologist

Alan Lyell Centre for Dermatology, Western Infirmary, Glasgow, G11 6NT, UK

A 56 year old man presented with a history of rapid onset hair 3 How can the change in hair colour be explained? thinning over one month. He also noted a reduced need to shave 4 What treatments can be offered for this condition? his . In addition, he felt that the colour of his hair had changed from dark brown with “an occasional grey” to white. Specifically, he described attending a Christmas party with Questions “normal” hair yet by Christmas Day his hair and beard were 1 What is the differential diagnosis? white. There was no loss of elsewhere. The was Short answer noted on brushing and in the shower basin. He denied , Hair loss can be caused by hair cycle disorders, inflammatory scale, or elsewhere. He had no history of serious illnesses conditions that damage the follicle, or inherited and acquired or stresses over the past year. His medical history included disorders of the hair shaft. The differential diagnosis for acquired hypertension for which he took longstanding atenolol and diffuse non-scarring alopecia includes , ramipril. Both his parents had good heads of hair in old age and underlying medical conditions (such as anaemia, thyroid disease, there was no family history of autoimmune conditions. chronic inflammatory conditions, and ), drug induced Examination found white hair with a remaining band of hair loss, and diffuse . Androgenetic alopecia, pigmented hair at the occiput (figure). He had diffuse thinning although classically more localised, should also be considered. of the hair and his beard was sparse, but there was no patchy In this case, the clinical presentation and histology were most hair loss, scale, , or scarring. Blood samples were in keeping with diffuse alopecia areata. obtained and punch biopsies of the scalp showed an increased proportion of follicles in catagen or telogen phase, accompanied Long answer by a peribulbar lymphocytic infiltrate with no evidence of Diffuse hair loss can be caused by any process that interrupts scarring. the normal hair cycle. Loss of in the telogen phase (or resting phase) is the most common, whereas hair loss in the anagen phase (or growth phase) is caused by chemotherapy or radiotherapy. In the normal hair cycle, people shed 50-150 telogen hairs a day.1 Anagen hair loss is always related to disease. Disorders of hair loss can be divided into scarring and non-scarring alopecias. Non-scarring alopecias can be further subdivided depending on the distribution of hair loss—focal Photographs showing evidence of hair thinning and white (such as alopecia areata caused by secondary and hair, with a band of pigmented hair remaining only at the posterior scalp ), patterned (androgenetic alopecia in men, female , and ), and diffuse ( after chemotherapy, telogen effluvium, loose Questions anagen syndrome in children, and diffuse alopecia areata). The most common cause of acquired diffuse non-scarring hair 1 What is the differential diagnosis? loss is telogen effluvium. In this condition anagen hair follicles 2 What investigations should be performed in patients with prematurely transition into telogen, resulting in increased hair loss? shedding of the hairs at the end of telogen two to three months later. Causes of telogen effluvium include physiological stress

Correspondence to: R C Lamb [email protected]

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(such as fever, systemic illness, , and ); Long answer emotional stress; and medical conditions, including renal failure, A thorough history can provide valuable clues for diagnosis and thyroid disease, inflammatory bowel disease, and chronic 2 help identify risk factors for hair loss, including medical history infections such as HIV and secondary syphilis. of illness, current drugs and family history of alopecia areata, Other causes of diffuse hair loss include nutritional deficiency, androgenetic alopecia, atopy, or autoimmune conditions. such as iron or . Chemotherapeutic drugs cause Examination may identify exclamation mark hairs in acute anagen hair loss, but other drugs can cause telogen hair loss. alopecia areata, scarring or non-, erythema or These include oral contraceptive, retinoids, angiotensin scale indicating an inflammatory condition of the scalp, or converting enzyme inhibitors, β blockers (which could be patterned hair loss with miniaturisation of hairs in androgenetic relevant in this case), and anticoagulants such as warfarin and alopecia. 2 heparin. Patterned hair loss or androgenetic alopecia in men The hair pull test can be performed to identify active hair loss. usually occurs at the vertex and frontal hairline so is more In telogen effluvium, thinning of the hair involves the entire localised than seen here. Nonetheless, androgenetic loss should scalp and may also be noted on other hairy regions of the body be considered in this case because of relative sparing of the (such as the beard). A gentle hair pull in telogen effluvium may occipital hair (figure). However androgenetic alopecia would be positive for two or more hair shafts per pull; these will be not affect other hair bearing skin, such as the beard area, and normal telogen hairs on microscopic examination, and the miniaturisation of the hair follicles and hair shafts of different telogen “bulb” or “club hair” will be identifiable diameters would be seen on examination. macroscopically. A forcible hair pluck (or trichogram) will show Alopecia areata is a cause of anagen hair shedding and a mixture of normal anagen and telogen hairs, but with more classically causes patchy hair loss with a sharply demarcated than 20% of hairs being in the telogen phase. The hair pull test edge. However, it may also be seen as diffuse hair loss, complete in androgenetic alopecia may be positive in the region affected hair loss of the scalp (), or complete loss of scalp by the disease but negative away from affected areas. and body hair (). Prevalence is reported to 3 Scalp biopsy is also useful. It is not routinely performed to be 0.1% and the lifetime risk is 2%. Patients are often unaware diagnose alopecia areata and other non-scarring causes of hair of the time period over which hair loss occurred, but loss but was done at the patient’s request in this instance. occasionally loss is rapid, as was the case here, and patients can Performing two punch biopsies allows for horizontal and vertical be more precise about timing. Associated changes are sections. Histologically, telogen effluvium is characterised by possible, including pitting and trachyonychia (“sandpaper normal total number of hairs, normal number of terminal hairs, nails”). Atopy and autoimmune conditions are also associated increased telogen counts (>20%), and the absence of with alopecia areata. The course of the condition is unpredictable and scarring. Androgenetic alopecia is but 34-50% of patients with patchy alopecia areata experience characterised by normal total number of follicles, increased spontaneous regrowth within 12 months. Risk of recurrence, 4 number of vellus hairs, numerous follicular fibrous tracts, and however, is high. The loss of immune privilege has recently absence of inflammation, whereas unaffected occipital scalp be suggested to be central to the development of alopecia areata. looks normal. Acute alopecia areata is characterised by normal The normal proximal follicular epithelium of the human hair total number of follicles, increased number of catagen and follicle does not express MHC class I or II antigens and is telogen hairs, and the presence of a peribulbar inflammatory therefore immune privileged, whereas in alopecia areata these infiltrate that mainly affects terminal anagen and catagen hair antigens are expressed, resulting in the loss of immune 6 5 bulbs. By comparison, in scarring alopecias a band-like privilege. lymphocytic inflammatory infiltrate is seen initially, with It is also important to recognise that more than one disease subsequent fibrosis, a decreased total number of hairs, and loss process may be at play. For example, this patient might have a of sebaceous glands. degree of androgenetic alopecia as well as telogen loss secondary Dermoscopy (or trichoscopy) is also used by some specialists to a drug (such as atenolol), which would cause diffuse thinning to examine follicular ostia and individual hairs, and it can be a with some sparing of the occipital hair. These diagnoses do not, useful diagnostic tool.7 however, account for the colour change. Follicular , which can coexist with other autoimmune disorders such as Laboratory investigations should include full blood count and alopecia areata, could be contributing to the colour change. serum ferritin to identify anaemia and iron deficiency, thyroid function testing, and baseline liver and kidney tests to rule out Overall in this case the examination findings, clinical picture, underlying chronic disease. Diffuse hair loss can be caused by and histopathological appearances were most in keeping with infections, such as HIV or syphilis, so screening should be diffuse alopecia areata, although coexistent androgenetic considered in high risk patients. In addition, serum alopecia or telogen effluvium (or both) remained a possibility concentrations of total and free , sex hormone at presentation. binding globulin, and should be checked. Prostate specific antigen should also be checked if indicated. 2 What investigations should be performed in patients with hair loss? 3 How can the change in hair colour be Short answer explained? Laboratory investigations should include full blood count and Short answer serum ferritin to identify anaemia and iron deficiency, thyroid Pigmented hair is preferentially targeted in alopecia areata, function testing, and baseline liver and kidney tests to rule out whereas white hairs are spared, giving the appearance of colour underlying chronic disease. Diffuse hair loss can be caused by change. It is thought that this may be due to the presence of infections such as HIV or syphilis, so high risk patients should antibodies to melanocytes within pigmented hairs.8 be screened.

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Long answer with side effects such as itch and lymphadenopathy, and the Pigmented hair is preferentially targeted in alopecia areata, area must not be exposed to ultraviolet radiation for at least 24 whereas white hairs are spared, giving the appearance of colour hours. Response rates are highly variable, ranging from 6% to 85%, so a course of DCP treatment represents a considerable change. Sudden whitening of the hair in acute extensive alopecia 13 areata is known as “total canities” or “Marie Antoinette commitment on the part of the patient. syndrome.”9 It is thought that this may be due to the presence Other treatments described in the literature include topical of antibodies to melanocytes within pigmented hairs.8 In (2% or 5%), topical irritants (such as dithranol, addition, initial regrowth in alopecia areata will be with tazarotene, azelaic acid), topical or oral photochemotherapy, non-pigmented hairs, possibly secondary to reduced photodynamic therapy, pulsed oral , and other melanoblasts and abnormal melanogenesis in areas of hair immunosuppressants. 10 regrowth. This phenomenon is especially common in localised Because hair loss can be associated with serious psychological disease but pigmented hairs usually begin to appear after anagen morbidity, patients may benefit from support groups such as phase. Alopecia Awareness (www.alopecia-awareness.org.uk), the Follicular vitiligo is also a possibility. This condition causes prescription of , and permanent tattooing of . loss of hair pigment but there may also be other pigment changes on the body and hair thinning is absent. Patient outcome 4 What treatments can be offered for this Biopsy confirmed that the patient had diffuse alopecia areata, condition? and six months after the onset of hair loss (and with no treatment) his hair is beginning to regrow and repigment in Short answer several areas, which is suggestive of spontaneous resolution. Diffuse alopecia areata can be difficult to treat. Spontaneous The patient therefore does not currently want active treatment. regrowth is reported in half of cases. Treatments include topical immunotherapy and intralesional or topical corticosteroids. Competing interests: We have read and understood the BMJ Group Because hair loss can be associated with serious psychological policy on declaration of interests and declare the following interests: morbidity, patients may benefit from support groups such as None. Alopecia Awareness (www.alopecia-awareness.org.uk) and the Provenance and peer review: Not commissioned; externally peer prescription of wigs. reviewed. Patient consent obtained. Long answer Diffuse alopecia areata can be difficult to treat and has cosmetic 1 Paus R, Cotsarelis G. The biology of hair follicles. N Engl J Med 1999;341:491-7. 2 Sperling LC, Jorizzo JL. Hair disorders in systemic disease. In: Callen JP, Jorizzo JL, and psychological implications for the patient. Spontaneous eds. Dermatological signs of internal disease. 3rd ed. Saunders, 2003:315-21. regrowth is reported in half of cases. Acute diffuse alopecia 3 Safavi K. Prevalence of alopecia areata in the first national health and nutrition examination survey. Arch Dermatol 1992;128:702. areata (as seen in this patient) has recently been reported to be 4 Messenger AG, McKillop J, Farrant P, McDonagh AJ, Sladden M. British Association of associated with a more favourable prognosis than chronic Dermatologists’ guidelines for the management of alopecia areata 2012. Br J Dermatol 11 2012;166:916-26. disease. 5 Paus, R, Slominski, A, Czarnetzki, BM. Is alopecia areata an autoimmune-response Treatments are those used in localised disease, including topical against melanogenesis-related proteins, exposed by abnormal MHC class I expression in the anagen hair bulb? Yale J Biol Med 1994;66:541-54. immunotherapy with diphencyprone acetate (DCP) and super 6 Wener B, Mulinari-Brenner F. Clinical and histological challenge in the differential diagnosis potent topical or intralesional (5-10 mg/mL of diffuse alopecia: female androgenetic alopecia, telogen effluvium and alopecia areata. Part I. An Bras Dermatol 2012;87:742-7. triamcinolone acetonide injected every four to eight weeks into 7 Miteva M, Tosti A. Hair and scalp dermoscopy. J Am Acad Dermatol 2012;67:1040-8. the mid-: 0.05-0.1 mL will produce a tuft of hair growth 8 Tobin DJ, Paus R. Graying: gerontobiology of the pigmentary unit. Exp Gerontol 4 2001;36:29-54. about 0.5 cm diameter ). However, owing to the nature of diffuse 9 Navarini AA, Nobbe S, Trueb RM. Marie Antoinette syndrome. Arch Dermatol disease these treatments can be more difficult to administer than 2009;145:656. 10 Mandani S, Shapiro J. Alopecia areata update. J Am Acad Dermatol 2000;42:549-66. for localised disease. 11 Lew BL, Shin MK, Sim WY. Acute diffuse and total alopecia: a new subtype of alopecia DCP is a potent contact sensitiser that is applied to the scalp areata with a favourable prognosis. J Am Acad Dermatol 2009;60:85-93. 12 Gilhar A, Etzioni A, Paus R. Alopecia areata. N Engl J Med 2012;366:1515-25. with the aim of causing inflammation and stimulating hair 13 Rokhsar CK, Shupack JL, Vafai JJ, Washenik K. Efficacy of topical sensitizers in the regrowth. The mechanism of action is unknown, but it has been treatment of alopecia areata. J Am Acad Dermatol 1998;39:751-61. described as an immune deviation strategy.12 However, treatment with DCP is time consuming, requiring once weekly visits to Cite this as: BMJ 2013;347:f6112 hospital for at least six months. In addition, DCP is associated © BMJ Publishing Group Ltd 2013

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