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Cicatricial (scarring) alopecia: an overview and a clinical approach to diagnosis /DERMQUEST.COM GALDERMA/DERMQUEST.COM GALDERMA/DERMQUEST.COM GALDERMA/DERMQUEST.COM Yi Zhen Chiang, Firas Al-Niaimi Figure 1. Lichen planopilaris (LPP). Figure 2. Figure 3. Dissecting of the Figure 4. keloidalis nuchae (PB). scalp (DCS). (AKN). Scarring alopecia, also known as cicatricial alopecia, forms an important group of disorders whereby there is irreversible damage to hair follicles resulting in scarring and permanent . The hair follicles can be conditions appear more commonly in in pigmentation, scalp bogginess, FFP is a variant of LPP and presents destroyed directly by a primary process (primary cicatricial alopecia) or secondary to an underlying disease certain racial groups and age (Han, 2006). pustules, crusting) typically with a band-like symmetrical or an external agent (secondary cicatricial alopecia). A diagnosis of scarring alopecia can be achieved on A complete and focused history should 8 Other area(s) of hair loss recession of the frontal hairline. aim to elicit the following points: 8 Signs of skin disease elsewhere (eg, The condition is common in post- both clinical and histological grounds. Patients with cicatricial alopecia often have significant psychosocial 8 Ethnic origin , erythematosus). menopausal women. GLS is another impact and nurses can play an important role in the care for these patients. 8 Age variant of LPP and is characterised by 8 Onset Investigations the triad of patchy, progressive scarring Introduction exact pathogenesis of primary cicatricial The classification is based primarily on the 8 Progression Samples for , culture and alopecia, non-scarring alopecia of axillary Scalp hair is an important component of alopecias is not known, but is likely to predominant inflammatory infiltrate found 8 Local and systemic symptoms sensitivity (bacterial and fungal) are and pubic hair, and widespread horny identity, body image and self-esteem, and result from an irreversible damage to the on scalp biopsy, as shown in Table 1 (Olsen, 8 Other medical problems (eg, presence useful if there are signs of infection such follicular () on is often used to express personality and epithelial stem cells that reside 2003). of autoimmune diseases or associated as crusting, scaling, pustules or scalp trunk and limbs (Ghislain, 2006). sexuality. It is therefore not surprising that in the hair follicle ‘bulge’. This is suggested inflammatory skin conditions such bogginess. Pustules, however, are not alopecia (hair loss) can lead to significant by the pattern of seen in Clinical assessment as lupus erythematosus (LE), lichen always associated with infections, and can Chronic cutaneous lupus erythematosus psychosocial distress, psychiatric disorders, cicatricial alopecia, which is around the Patients with cicatricial alopecia often planus (LP), infections, malignancies) be seen in normal scalps (Harries, 2009). A third of the cases of CCLE have scalp marital problems and career-related ‘bulge’, as opposed to non-cicatricial present fairly late due to the subtle, 8 Previous treatment (including any involvement. This condition is more problems (Hunt, 2005). Alopecia can alopecia, which is around the hair bulb relentless progression of the disease. previous exposure to thermal burns, A scalp biopsy is often essential in common in adult women. It commonly be cicatricial (scarring) or non-cicatricial (Harries, 2009). Diagnosis can be challenging due to the radiation treatment) the assessment of cicatricial alopecia. It affects the central scalp with associated (non-scarring). This review will focus on evolving clinical and histological features 8 Previous hair care practice (eg, use of will help to confirm scarring (if there skin changes such as , cicatricial alopecia with an aim to providing Secondary cicatricial alopecia develops over time, and the overlapping clinical hot combs, excess traction) is any clinical doubt), and to identify scalp, follicular plugging, change in a systematic approach to the assessment as a result of an underlying process or and histological features seen in one the secondary cause of cicatricial pigmentation, and skin atrophy (Amnessi, of patients with suspected cicatricial an external agent. Potential causes are condition with the other (Harries, 2009). Examination alopecia, or to confirm the diagnosis of 1999). alopecia and an overview of the different inflammatory/autoimmune diseases (eg, Nevertheless, a systematic approach to A crucial first step in clinical examination a primary cicatricial alopecia based on types of cicatricial alopecia. , sarcoidosis), infections (eg, establish an accurate diagnosis is a vital first is to confirm the presence of scarring the predominant inflammatory infiltrate Pseudopelade of Brocq ), neoplastic processes (eg, step in successful management. on the areas of hair loss, which is involved (as shown in Table 1). It is still not clear whether PB is a disease Cicatricial alopecia forms a rare, primary skin cancer, metastatic carcinoma), characterised by the loss of follicular ostia in its own right or just the end stage of but important group of disorders and physical agents (eg, ionising radiation, History (openings). Additional clues to scarring Diagnosis a different scarring process. However, characterised by permanent destruction thermal burn) (Headington, 1996). Patients with cicatricial alopecia may are epidermal atrophy, irregularly spaced The major forms of primary cicatricial it has a characteristic clinical picture. of hair follicles, resulting in scarring present with an acute or gradual onset hair shafts and hair tufting (multiple alopecia are lichen planopilaris (LPP) Typically, there are multiple, variably and permanent hair loss. The hair Classification of hair loss and symptoms. Common hairs emerging from a single follicular (Figure 1) and variants — frontal fibrosing sized, white to skin-coloured plaques follicle can be destroyed by a primary The North American Hair Research symptoms include pain, irritation, itching ostium) (Han, 2006). A good light, aided alopecia (FFA) and Graham-Little on the vertex (known as ‘footprints process aimed directly at the hair follicle Society (NAHRS) has produced a working and discharge. A full history should include by a magnifying lens or dermatoscope, is syndrome (GLS); chronic cutaneous lupus in the snow’), and they are largely (primary cicatricial alopecia (PCA)), or classification of primary cicatricial alopecia. the patient’s ethnic origin and age as some essential in the examination of the scalp. erythematosus (CCLE); pseudopelade of asymptomatic (Braun-Falco, 1986, secondary to a generalised destructive Brocq (PB) (Figure 2); central centrifugal Dawber, 1992). process within the skin, which ultimately Scalp inflammation often indicates cicatricial alopecia (CCCA); destroys the hair follicle’s capacity for Table 1. active disease, and the signs of an active decalvans (FD); dissecting cellulitis of the Central centrifugal cicatricial alopecia regeneration (secondary cicatricial The North American Hair Research Society classification of primary cicatricial disease include erythema, scaling, crusting, scalp (DCS) (Figure 3); CCCA is often seen in women of alopecia) (Harries, 2009). alopecias (Olsen EA, 2001). pustules, scalp bogginess, and a positive (TF); and (AKN) African descent and has been referred pull test with anagen bulbs seen on the (Figure 4) (Ross, 2005). to as ‘follicular degeneration syndrome’ The of PCA has Inflammatory infiltrate Diagnosis hair mount (Harries, 2009, Han, 2006). or ‘hot comb alopecia’ (Sperling, 1992). been reported to range from 3.2% to Lichen planopilaris and variants The disorder commonly starts over Lichen plano pilaris and variants; chronic cutaneous lupus erythematosus; 7.3% (Whiting, 2001, Tan, 2004). The Lymphocytic The following features should be LPP is characterised by multiple patches of the vertex and spreads symmetrically pseudopelade of Brocq; central centrifugal cicatricial alopecia recorded: permanent alopecia distributed over the and centrifugally. The hair loss is often 8 Pattern of hair loss and extent of scalp central scalp, mostly affecting middle-aged incomplete with groups of hairs Yi Zhen Chiang is a Specialty Trainee in Neutrophilic /Tufted folliculitis; dissecting cellulitis of the scalp Dermatology at Birmingham City Hospital. involvement women. Mild to moderate itching has been remaining in the area of scarring. Firas Al-Niaimi is a Fellow in Mohs Surgery Mixed Folliculitis acne keloidalis 8 Presence or absence of scarring reported. Scaling and erythema around Symptoms are usually absent and there and , St John’s Institute of Dermatology, Non-specific End stage of scarring 8 Associated skin changes on the scalp the follicles in the expanding areas of is no clinical evidence of inflammation London (eg, erythema, scaling, atrophy, change alopecia are commonly seen (Ross, 2005). (Harries, 2009).

32 Dermatological Nursing, 2012, Vol 11, No 4 www.bdng.org.uk www.bdng.org.uk Dermatological Nursing, 2012, Vol 11, No 4 33 Clinical REVIEW

Folliculitis decalvans, tufted folliculitis, management of these patients should dissecting cellulitis of the scalp and acne also include addressing their psychosocial Table 2. keloidalis nuchae aspects. Patients can be referred to a Useful resources for patients and These conditions form a group of scarring psychologist for counselling and ways healthcare professionals. alopecia that present with discharging to deal with the psychological impact of material. They affect men and women hair loss if needed. Regular monitoring Alopecia UK Online www.alopeciaonline.org.uk from young adulthood to middle-age. The of disease progression and adequate Alopecia Help and Advice (Scotland) www.alopeciascotland.co.uk vertex is most commonly affected. The provision of information can help relieve Bebold Alopecia Awareness www.bebold.org.uk scarred areas are often hypertrophic with unnecessary . Dermatology nurses Changing Faces www.changingfaces.org.uk features of pustules, crusting and tufts. TF is who care for these patients can play a Cicaticial Alopecia Research Foundation often thought to be a variant of FD. DCS is role in providing continuous support, www.carfintl.org seen almost exclusively in males of African information on scalp and hair care Hair Foundation www.hairfoundation.org descent. There are typically multiple painful practice, techniques of scalp coverage Hairline International www.hairlineinternational.com interconnecting scalp nodules, fluctuant and camouflage, and to ensure that these Hunt N, McHale S (2004) Coping with alopecia. nodules which may discharge purulent patients have a good understanding London: Sheldon Press, material, and the presence of sinus tracts about the nature and treatment of their Top 50 Blogs for Understanding and Coping with (Brooke, 2001). AKN commonly involves condition. Useful information on cicatricial Alopecia http://msndegree.com/top-50-blogs-for- the occipital scalp. Like DCS, AKN tends to alopecia can be obtained from the British understanding-coping-with-alopecia Outlook www.nbt.nhs.uk/depts/SurgicalServices/ affect young men of African descent. Association of Dermatologists (BAD) Outlook plaques are seen, with secondary infection website and various patient support suggested by the presence of pustule groups (Table 2) (Hunt, 2005). formation, pain and discharge (Sperling, Han A, Mirmirani P (2006) Clinical approach 2000, Dinehart, 1989). Conclusion to the patient with alopecia. Semin Cutan Med Cicatricial alopecia forms an important Surg 25(1): 11-23 Management group of disorders that result in scarring Harries MJ, Sinclair RD, MacDonald-Hull S, et Management of cicatricial alopecia is and permanent hair loss. An early diagnosis al (2008) Management of primary cicatricial challenging as hair regrowth in scarred can be achieved by a systematic approach alopecias: options for treatment. Br J Dermatol 159(1): 1-22 areas hardly ever occurs. The principle to the clinical assessment. Patients with aim of treatment is to reduce symptoms cicatricial alopecia often have significant Harries MJ, Trueb RM, Tosti A, et al (2009) and to stop or slow progression of the psychosocial impact and management of How not to get (r)ed: pointers to the correct diagnosis in patients with suspected scarring process. It is important to ensure these patients should address both their primary cicatricial alopecia. Br J Dermatol that patients understand the aims of their physical and psychological aspects. DN 160(3): 482-501 treatment and are provided with a realistic Headington JT (1996) Cicatricial alopecia. expectation. Dermatol Clin 14(4): 773-82 References As a general rule, immunosuppressants Hunt N, McHale S (2005) The psychological such as potent topical corticosteroids, Amnessi G, Lombardo G, Gobello T, Puddu P impact of alopecia. BMJ 331: 951-3 (1999) A clinicopathologic study of scarring intralesional triamcinolone acetate, oral alopecia due to lichen planus: comparison Ross EK, Tan E, Shapiro J (2005) Update corticosteroids, and antimalarials (eg, with scarring alopecia in discoid lupus on primary cicatricial alopecias. J Am Acad ) are useful for the erythematosus and pseudopelade. Am J Dermatol 53(1): 1-37 lymphocyte-predominant sub-group Dermatopathol 21: 324-31 Sperling LC, Sau P (1992) The follicular of primary cicatricial alopecia; while Braun-Falco O, Imai S, Schmoeckel C, Steger degeneration syndrome in black patients. ‘Hot antimicrobials (eg, ) and O, Bergner T (1986) Pseudopelade of Brocq. comb alopecia’ revisited and revised. Arch Dermatol 128(1): 68-74 are useful for the - Dermatologica 172(1): 18-23 predominant sub-group. Patients with Brooke RC, Griffiths CE (2001) Folliculitis Sperling LC, Homoky C, Pratt L, Sau P (2000) completely stable or ‘burned-out’ cicatricial decalvans. Clin Exp Dermatol 26(1): 120-12 Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol 136(4): 479-84 alopecia may benefit from scalp reduction Dawber R (1992) What is pseudopelade? Clin surgery and, possibly, hair transplantation Exp Dermatol 17(5): 305-6 Olsen EA, Bergfeld WF, Cotsarelis G, et al (Harries, 2008). Damaging hair care (2003) Summary of North American Hair Dinehart SM, Tanner L, Mallory SB, Herzberg Research Society (NAHRS)-sponsored practice such as tight hair braiding, use AJ (1989) Acne keloidalis in women. Cutis workshop on cicatricial alopecia, Duke of chemical relaxers, glued-in or sewn- 44(3): 250-2 University Medical Center, February 10 and 11, in hair weaves should be stopped. The 2001. J Am Acad Dermatol 48(1): 103-10 de Berker D, Dissaneyeka M, Burge S (1992) underlying cause of a secondary cicatricial The sequelae of chronic cutaneous lupus Tan E, Martinka M, Ball N, et al (2004) Primary alopecia should be identified and treated erythematosus. Lupus 1(3): 181-6 cicatricial alopecias: clinicopathology of 112 accordingly. cases. J Am Acad Dermatol 50(1): 25-32 Ghislain PD, van Eeckhout P, Ghislain E (2006) Graham-Little-Piccardi-Lasseur Whiting DA (2001) Cicatricial alopecia: As hair loss is associated with syndrome: a 20-year follow-up. Dermatology clinico-pathological findings and treatment. significant psychosocial morbidity, 206: 391-2 Clin Dermatol 19(2): 211-25

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