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Female Pattern Loss and its Relationship to Permanent/ Cicatricial Alopecia: A New Perspective

Elise A. Olsen Division of , Department of Medicine Duke University Medical Center, Durham, North Carolina, USA

Female pattern (FPHL) is a common hair disorder of the central . The clinical change in hair density, related to a change in the hair cycle and miniaturization of the , is generally considered to be potentially reversible. However, there is now evidence of a permanent hair loss that develops in a subset of women with FPHL. The presence of a perifollicular lymphohistiocytic infiltrate and fibrosis is seen without follicular drop-out in biopsies of women with FPHL and with a notable follicular drop-out in a cicatricial form of this condition (heretofore called cicatricial ) as well as in fibrosing alopecia in a pattern distribution, currently classified as a subset of lichen planopilaris. The potential relationship of these conditions as well as frontal fibrosing alopecia and central centrifugal cicatricial alopecia, two other conditions of permanent hair loss seen primary in women, is discussed. Key words: cicatricial alopecia/female pattern hair loss J Investig Dermatol Symp Proc 10:217 –221, 2005

Cicatricial alopecia is a poorly understood group of presents as flesh-toned patches of disorders, generally only diagnosed by dermatologists and hair loss with little, if any, clinical inflammation (Braun-Falco then usually only late in the process. The primary cicatricial et al, 1986; Brocq et al, 1905; Gay Prieto, 1955), but at least alopecias all initially involve some form of inflammation in in the early stages, may display histological evidence of the region of the follicular isthmus (and thus also the bulge inflammation (Newton et al, 1987). The patches of hair loss area) plus/minus inflammation in the infundibular area in the latter condition typically do not show significant (Headington, 1996; Whiting, 2001a; Bergfeld and Elston, atrophy but primarily loss of follicular ostia. Permanent 2003). Clinical inflammation, if present, may appear in the alopecia may also present without any clinical or histologi- early, but not the final stages and primarily in those types of cal inflammation as illustrated by the of cicatricial alopecia that involve the infundibulum such as atrichia with papular lesions (Zlotogorski et al, 2001). In this lichen planopilaris and chronic erythematosus. In the condition, the first pelage is normal but the hair is unable to late stage of cicatricial alopecia, regardless of cause, there transition from telogen to anagen after the first telogen and is loss of follicular ostia. In only some types of cicatricial thus the hair loss becomes permanent. Loss of follicular alopecia, including the majority of primary cicatricial ostia follows but not atrophy. alopecia, is the other hallmark of this general process There are other types of hair loss, generally considered present, i.e. epidermal atrophy. Because of the lack of fully reversible, that may, in certain situations, also become clinical atrophy and/or histological evidence of a ‘‘scar’’ in permanent. For example, long-standing / all patients who experience loss of the follicular apparatus, universalis (Whiting, 2001a, b) or extensive long-standing the umbrella term ‘‘permanent alopecia’’ (Bergfeld and baldness in male pattern hair loss (MPHL) (Headington and Elston, 1994) may be more appropriate than ‘‘cicatricial Novak, 1984), may develop follicular drop-out in the alopecia’’ when discussing the breadth of these disorders. affected areas. The affected scalp in these situations is It is very apparent that cicatricial, or permanent, alopecia usually devoid of any visible hair, including , and may have abundant histological evidence of inflammation has loss of follicular ostia on clinical exam. Neither of these without any clinical sign of inflammation. For example, conditions, however, typically has clinical signs of scalp chronic lupus erythematosus rarely may present with inflammation preceding the permanent hair loss. patches of hair loss without evidence of clinical inflamma- Female pattern hair loss (FPHL) is a condition character- tion and yet there is usually a heavy perifollicular lympho- ized clinically by a decrease in hair density in the central cytic infiltrate in the (Bergfeld and Elston, 2003). scalp and histologically by miniaturization of some follicles and an increased percentage of hair in telogen in the affected area (Olsen, 2001). ‘‘Patterning’’ in FPHL is much Abbreviations: CCCA, central centrifugal cicatricial alopecia; less obvious than in MPHL but may be one of three CPHL, cicatricial pattern hair loss; FAPD, fibrosing alopecia in a patterns: in order of frequency of occurrence, they are pattern distribution; FPHL, Female pattern hair loss; MPHL, male pattern hair loss frontal accentuation (‘‘Christmas tree’’ pattern), diffuse There are no conflicts of interest to disclose. central or vertex/frontal (male pattern) (Olsen, 2001). Most

Copyright r 2005 by The Society for Investigative Dermatology, Inc. 217 218 OLSEN JID SYMPOSIUM PROCEEDINGS patients with FPHL do not require a biopsy for confirmation of the diagnosis, however, as the clinical findings of a decrease in density of the central scalp hair with sparing of the occiput, no patches of hair loss, frontal accentuation of hair loss (if present), negative hair pull on the sides and back of the scalp and a normal appearing scalp are usually sufficient to make the diagnosis (Olsen, 1999). Although generally considered a fully reversible form of alopecia, there is histological evidence of inflammation and fibrosis in 70% of patients with FPHL (Whiting, 1998) and inflammation in MPHL has been shown to correlate with decreased responsiveness to topical (Whiting, 1993). Scant attention until recently has been paid to this component of FPHL, i.e. the perifollicular inflammation and fibrosis that may affect the potential for regrowth in this disorder (Whiting, 1998). In the last 4 y, however, a distinct type of cicatricial alopecia typified by inflammation and fibrosis with hair loss in the distribution of typical FPHL has Figure 1 been reported i.e., ‘‘fibrosing alopecia in a pattern distribu- Frontal fibrosing alopecia. (Reprinted with permission from Bergfeld tion’’ (FAPD) (Zinkernagel and Trueb, 2000). In addition, a and Elston (2003) Courtesy of Dr Steven Kossard.) common form of cicatricial alopecia that occurs in African American women i.e., central centrifugal cicatricial alopecia male patient, the finasteride also decreased the clinical (CCCA) (Olsen et al, 2003), presents in the same area of the inflammation. These results speak to a potential - scalp as FPHL and also is typified by a perifollicular related etiology in this disorder. That an alone lymphocytic infiltrate. Frontal fibrosing alopecia is a recently should decrease scalp inflammation is an interesting described form of permanent alopecia whose is observation that needs further follow-up. similar to FAPD but whose distribution of loss occurs only in the frontotemporoparietal scalp (Kossard, 1994), not a distribution of hair loss typical of FPHL but one that Frontal Fibrosing Alopecia (FFA) overlaps with the frontal loss seen in some forms of MPHL. The purpose of this paper is to review the potential and Frontal fibrosing alopecia is a condition that was first established associations between FPHL and permanent described by Kossard (1994). This type of hair loss almost alopecia that occur in this same scalp distribution. exclusively presents in postmenopausal women although a few reports in younger women have been published (Faulkner et al, 2002; Bergfeld and Elston, 2003). A gradual Fibrosing Alopecia in a Pattern recession of not only the frontal but also the temporoparietal Distribution (FAPD) hairline follows perifollicular with or without associated follicular keratosis. The hair loss progresses Reported by Zinkernagel and Trueb (2000), FAPD generally backward to about 8 cm maximum (Kossard et al, 1997) (Fig presents as accelerated hair loss in the central scalp of 1). This recession occurs in a distribution of a male pattern subjects with underlying pattern hair loss. Females are more of frontal scalp hair loss (Norwood type IIa or Hamilton type commonly affected than men, the latter who all have had IV pattern of hair loss) (Olsen, 2003) although the degree of preceding male pattern baldness. Clinically, FAPD presents parietal hair loss is unusual for even MPHL. Moreover, with perifollicular erythema plus/minus follicular hyperker- perifollicular eythema is not typical for frontal recession in atosis and loss of follicular orifices in the central scalp. On MPHL. biopsy, there is a lymphohistiocytic infiltrate around the Venning and Dawber (1988) noted that 37% of post- isthmus and infundibular region of the hair follicle, con- menopausal women have temporal recession but did not centric perifollicular lamellar fibrosis, loss, note any cicatricial process. Hamilton (1951) described and a lymphocytic interface dermatitis with destruction of frontal hair loss in women with androgenetic alopecia the basal keratinocytes (Zinkernagel and Trueb, 2000). including frontotemporal recession extending to 3 cm These histological findings are identical to that seen in posterior to a coronal plane between the two external lichen planopilaris and thus despite its location and milieu in auditory meatus . Ludwig’s (1977) pattern of diffuse central which it develops, FAPD is currently classified as a subtype hair loss in women, on the other hand, always included of lichen planopilaris (Kossard, 1994; Sperling et al, 2000). retention of the frontal fringe of hair. The net result is a reduction in the number of Frontal fibrosing alopecia is characterized histologically follicles by either the underlying miniaturization process of by a lymphocytic infiltrate around the isthmus and infun- pattern hair loss or by replacement of follicles by fibrous dibular region of the hair follicles as well as concentric tracts. perifollicular lamellar fibrosis (Kossard, 1994). This histology Anti- in two women and finasteride in one man is identical to that of FAPD but has overlapping features with with FAPD have been reported to halt the progression of the ‘‘pseudopelade’’ and CCCA as well (Kossard et al, 1997). hair loss (Zinkernagel and Trueb, 2000). In the case of the Moreover, the presence of frontal fibrosing alopecia in 10 : 3 DECEMBER 2005 PERMANENT/CICATRICIAL ALOPECIA 219

Figure 3 Central centrifugal cicatricial alopecia. Figure 2 Cicatricial pattern hair loss. No inflammation of perifollicular preceded the loss of follicles in the central scalp. Central Center CCCA patients with FAPD (Zinkernagel and Trueb, 2000) raises the This common type of hair loss was first reported under the question of whether these two conditions, with histological term ‘‘ alopecia’’ by LoPresti et al (1968). This overlap, may be one fork in the road for FPHL towards a particular hair loss occurs almost exclusively in African cicatricial alopecia. FFA is currently classified as a variant of Americans, most commonly in African American women. As lichen planopilaris (Olsen et al, 2003). Of note, Tosti et al, it became apparent that African American men may also be (2005) has reported that frontal fibrosing alopecia, like effected as well as those individuals who have not used FAPD, may also respond to finasteride. hot combs (a method by which oiled or greased hair is straightened with a heated iron) (Sperling and Sau, 1992; Sperling et al, 1994; Headington, 1996), the terminology Cicatricial Pattern Hair Loss (CPHL) evolved to ‘‘follicular degeneration syndrome’’ per Sperling and Sau (1992) and more recently to CCCA (Olsen et al, The author has coined this term to describe the histologi- 2003), the descriptive term adopted by the North American cally confirmed presence of a permanent alopecia in the Hair Research Society (NAHRS). Whether hair grooming distribution of FPHL but without the perifollicular erythema methods specific to the African American culture, such as or follicular hyperkeratosis seen in FAPD (Fig 2). The typical hot combs, , tight , heavy extensions, and a patient with cicatricial pattern hair loss is over 40 y of age variety of oils and , could cause or at least and has small (pencil eraser size) areas of ‘‘focal atrichia’’ contribute to the hair loss remains an unsubstantiated (Olsen, 2001). Focal atrichia appears to be a relatively assumption. specific sign of this late onset, clinically obvious FPHL (E. A. The hair loss in CCCA begins in the central midline scalp Olsen, unpublished information). Scalp biopsy shows a and is slowly progressive centrifugally (Fig 3). The condition perifollicular lymphohistiocytic infiltrate surrounding the may progress to a Hamilton Norwood Type VI or VII pattern isthmus, sebaceous gland loss, and concentric lamellar or may only involve the top of the scalp. Inflammation may fibrosis. What component of this hair loss is reversible and or may not be obvious clinically. Perifollicular erythema or which therapies might be most useful have yet to be follicular keratoses are not typical findings. Histologically, a determined. Despite the negative results of finasteride in a perivascular and perifollicular lymphocytic infiltrate, con- general population of postmenopausal women with ‘‘andro- centric lamellar fibrosis, sebaceous gland loss as well as genetic alopecia’’ (Price et al, 2000), many of whom had premature disintegration of the internal root sheath is typical minimal clinical evidence of hair thinning, it is possible that (Whiting, 2001a, b). Granulomatous inflammation secondary patients with CPHL, like those of FAPD, may respond to to follicular rupture may also be seen (Sperling and Sau, or to finasteride. 1992). Recently, these histological findings have been given 220 OLSEN JID SYMPOSIUM PROCEEDINGS the moniker of ‘‘pseudopelade’’ which, unlike the entity is present and using the term ‘‘FPHL’’ of pseudopelade described by Brocq, does not typically versus androgenetic alopecia might help to unravel the have the clinical counterpart of discrete non-inflammatory conflict between the concept of androgen etiology and lack patches of hair loss. of response of all patients to antiandrogens (Olsen, 2001). In No effective therapy for CCCA has been definitively this author’s view, separating out the FPHL that begins in identified. Discontinuation of the use of hot combs, relaxers, middle age from that which develops post-puberty through and/or excessive heat, all factors that have been identified early in the third decade, and considering all hormonal as possibly causing this condition, has, in most cases, not factors that are changing in concert at the time of the hair led to cessation of progressive hair loss. Whether hair care loss, may be the most fruitful path to eventually unraveling products that are used to moisturize the hair and scalp the cause of this most common type of FPHL. We will likely could be etiological factors has never been addressed. Both find that the concept of ‘‘senescent alopecia’’ as presented bacterial and fungal scalp infections need to be searched by Kligman (1988) overlaps with this latter type of late-onset for and treated as these may be contributing factors. FPHL. Although anti-inflammatory medications such as topical The assumption that FPHL is always a potentially steroids and/or systemic antibiotics may slow hair loss in reversible disorder also needs reassessment based on the many cases, this author has not found them to be uniform- histological findings of at least a moderate lymphocytic ly effective treatments. Well-controlled clinical trials are inflammation/fibrosis in 435% of patients with pattern hair needed to address these issues. loss compared with 9% of controls and the decrease in Although it is clear that MPHL is less common and less number of total follicles in affected patients (Whiting, 1998). extensive in men of African versus European descent (Smith The presence of a lymphocytic cicatricial alopecia in an and Wells, 1964; Setty, 1970), there appears to be a further FPHL distribution in both Caucasian females (FAPD and decrease in the incidence of FPHL in African American CPHL) and African American females (CCCA) begs the compared with Caucasian women (E. A Olsen, unpublished question of whether the inflammation in FPHL is a primary data). Potential explanations for this disparity include a or secondary component of the hair loss and how it relates difference in the hereditary potential for pattern hair loss in to the cicatricial component. The fact that antiandrogens or African American women compared with Caucasian wo- 5 aR inhibitors have been shown in limited cases to men, different hormonal characteristics in the two races, an decrease the inflammation and/or the clinical findings in intrinsically different clinical presentation of FPHL in African FAPD and frontal fibrosing alopecia begs consideration of American women or another hair loss process super- the potential role of androgens in the development of the imposed on FPHL in African American women that inflammatory reaction in the scalp. obliterates the usual non-scarring presentation. The possi- Other potential factors to explore in the etiology of the bility that women with CCCA could have underlying FPHL, inflammation seen in women with a central pattern of hair albeit with a degree of superimposed inflammation and loss typical of FPHL are hair care and scalp products and fibrosis not typically seen in Caucasian women with FPHL, grooming practices. Certainly Caucasian as well as African deserves consideration (Olsen, 2003). A controlled trial of American women are exposed to chemicals placed on the antiandrogens or 5 aR inhibitors early in the process may be hair and scalp (permanents and/or relaxers) and scalp heat of value to help sort out whether there is any androgen (hot curlers, hot combs, handheld, and/or turban dryers) relationship to this process. that may cause tissue damage and inflammation and contribute to hair loss. The effect of these common practices on the etiology of any permanent hair loss seen Discussion and Conclusions in FPHL needs to be sorted out. That the portion of the follicle in the bulge region, where a population of follicular FPHL, despite the frequency in the general population, stem cells lie, may be a particularly attractive target for remains a poorly understood disorder. At the present time, inflammatory cytokines has been shown in patients with in this author’s view, FPHL should be thought of as a clinical graft versus host disease where there appears to be some phenotype of central hair loss that may be caused by (1) a selective targeting of the undifferentiated stem cells of hereditary androgen-driven condition that presents in young the hair follicle (Sale et al, 1994). Furthermore, IL-1a,a women, the female counterpart of MPHL, (2) an age-related proinflammatory cytokine, has been shown to inhibit hair condition that may or may not have a hereditary component growth and fiber production in whole organ culture albeit and is likely related to a confabulation of hormonal changes with interindividual differences (Harmon and Nevins, 1993 resulting from the menopause, adrenopause and somato- and Mahe, Buan, Billoni et al, 1996). pause that are temporally related, (3) a non-hereditary In conclusion, FPHL is a clinical phenotype with most androgen driven process that may occur in any age patient. likely various etiologies all of which are potentially over- Up until recently, the view was that the central Ludwig lapping. Although classified as a non-scarring or reversible pattern of hair loss was the dominant clinical presentation of disorder, FPHL has the potential for transitioning to a FPHL and women who presented with this pattern of hair permanent type of hair loss. The role of inflammation in loss, regardless of age, had an androgen-driven genetic FPHL as a precursor to permanent hair loss (CPHL) or even process also called androgenetic alopecia (Frieden and to the reversible component of the hair loss needs further Price, 1986). This viewpoint was first challenged in 1999 exploration and the role that androgens and external factors when it was suggested that thinking of this presentation of play in the development of inflammation evaluated. In hair loss first by age of onset and second by whether or not particular, whether the condition of CCCA represents a 10 : 3 DECEMBER 2005 PERMANENT/CICATRICIAL ALOPECIA 221 more severe variant of CPHL in African Americans needs Mahe YF, Buan B, Billoni N, et al: Pro-inflammatory cytokine cascade in consideration. plucked hair. Skin Pharmacol 9:366–375, 1996 Newton RC, Hebert AA, Freese TW, Solomon AR: Scarring alopecia. Dermatol Clin 5:603–618, 1987 Olsen EA: The midline part: An important physical clue to the clinical diagnosis DOI: 10.1111/j.1087-0024.2005.10109.x of androgenetic alopecia in women. J Am Acad Dermatol 40:106–109, 1999 Manuscript received September 20, 2004; revised November 17, 2004; Olsen EA: Female pattern hair loss. J Am Acad Dermatol 45:S70–S80, 2001 accepted for publication December 2, 2004 Olsen EA: Pattern hair loss. In: Olsen EA (ed). Disorders of Hair Growth: Diagnosis and Treatment. New York: McGraw-Hill, 2003 Address correspondence to: Elise A. 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