Primary cicatricial alopecias: Clinicopathologyof 112 cases

Eileen Tan, MD,a,� Magdalena Martinka, MD, FRCPC,b Nigel Ball, MD, FRCPC,b

and Jerry Shapiro, MD, FRCPCa

Vancouver, British Columbia, Canada

Background: Cicatricial alopecias represent a diverse group of diseases characterized by a lack offollicular ostia and irreversible alopecia. There is limited literature on the epidemiology and therapeutics ofcicatricial alopecias.

Objective: The aim of this study was to review the epidemiology, clinical characteristics, and treatment ofinflammatory cicatricial alopecias in a mixed ethnic population referred to a university hair clinic.

Methods: The study population consisted of 112 patients seen during a 5-year period with acquiredprimary cicatricial alopecias. This represented 3.2% of the total number of trichologic consultations seen atthe University of British Columbia Hair Clinic, Vancouver, British Columbia, Canada.

Results: The ratio of lymphocytic to neutrophilic cicatricial alopecias was 4:1. Lymphocytic cicatricialalopecias had a tendency to affect middle-aged women, whereas neutrophilic cicatricial alopecias had apredilection for middle-aged men.

Conclusions: An accurate diagnosis of cicatricial alopecia is achieved through careful clinicopathologicevaluation. We suggest that a scalp biopsy is mandatory in all cases. Multiple biopsies may be necessary forsome affected individuals to achieve a definitive diagnosis as a result of a highly variable clinical course. Anaggressive multiple modality therapeutic approach is often necessary to prevent further irreversible follic-ular destruction, implying cicatrical alopecia should be considered a trichologic emergency. Currenttherapeutic options for lymphocytic cicatricial alopecia include corticosteroids, antimalarials, and isotreti-noin versus antibiotics, corticosteroids, and isotretinoin for neutrophilic cicatricial alopecias. (J Am AcadDermatol 2004;50:25-32.)

C icatricial (scarring) alopecias represent a di-verse group of diseases characterized bylack of follicular ostia and irreversible alope-

cia.1-3 Both adults and children are affected.2,3 Thereis limited literature on the epidemiology and thera-peutics of primary cicatricial alopecias.1 The objec-tive of this study was to review the epidemiology,clinical characteristics, and treatment modalities ofprimary cicatricial alopecias in a mixed ethnic pop-

ulation referred to a university hair clinic. We de-fined primary cicatricial alopecia as the preferentialdestruction of follicular epithelium and or its asso-ciated adventitial dermis with relative sparing of theinterfollicular reticular dermis.

The study population consisted of 112 patientswith primary cicatricial alopecias seen during a5-year period. Currently, various classificationschemes are available for cicatricial alopecias. Ac-cording to the North American Hair Research Society(NAHRS) working classification on cicatricial alope-

From the Division of Dermatologya and Department of Pathology,b

University of British Columbia, Vancouver General Hospital.Funding sources: The Canadian Hair Research Foundation.Disclosure: Dr Shapiro is a consultant for Pfizer and Merck.Accepted for publication April 23, 2003.Reprint requests: Jerry Shapiro, MD, FRCPC, UBC Hair Research and

Treatment Center, 835 W 10th Ave, Vancouver, BC, V5Z 4E8Canada. E-mail: shapiro@interchange.ubc.ca.

*Dr Tan is currently practicing in Singapore.0190-9622/$30.00Copyright © 2004 by the American Academy of Dermatology, Inc.doi:10.1016/j.jaad.2003.04.001

Abbreviations used:

AGA: androgenetic alopeciaCCLE: chronic cutaneous lupus erythematosusDC: dissecting cellulitisFD: follicultis decalvansLPP: lichen planopilarisNAHRS: North American Hair Research SocietyUBC: University of British Columbia, Vancouver,

British Columbia, Canada

25

cia,1 primary cicatricial alopecia can be divided intothe following categories: lymphocytic group (eg,chronic cutaneous lupus erythematosus [CCLE], li-chen planopilaris [LPP], classic pseudopelade[Brocq], central centrifugal cicatricial alopecia); neu-trophilic group (eg, folliculitis decalvans [FD], dis-secting cellulitis [DC]); and mixed group (eg, follic-ulitis keloidalis). There is a nonspecific cicatricialalopecia group defined as idiopathic scarring alope-cia with inconclusive clinical and histopathologicfindings. We did not classify any of our cases asnonspecific.

MATERIAL AND METHODSCase records of patients with primary cicatricial

alopecias, seen at the University of British Columbia(UBC) Hair Research and Treatment Center, Van-couver, British Columbia, Canada, were studiedfrom 1997 to 2001. The vast majority of referralswere from dermatologists, but there were referralsfrom family physicians and rheumatologists. Mostpatients lived within the province of British Colum-bia, Canada. Data including sex, ethnic group, ageof onset, clinical pattern, and treatment were re-corded. The ethnic distribution of clinic attendeeswas approximately as follows: 59.5% European-Cau-casian; 25% Oriental; 15% East Indian; and 0.5%African American. Scalp biopsies, at the advancingedge of the scarring lesion, were performed in allcases except those with obvious folliculitis keloida-lis. In addition, periodic acid–Schiff staining wasperformed (found to be negative in all biopsy spec-imens). Direct immunofluorescence was carried outin 68 patients. The final diagnosis of cicatricial alo-pecia was based on clinicopathologic correlation.

Cases were classified as CCLE if the prominentclinical findings were erythema, telangiectasia, andfollicular hyperkeratosis with the typical histologicpicture of lupus. A positive direct immunofluores-cence for the lupus band test was not necessary forthe diagnosis of lupus but helped to support thediagnosis if it was positive. LPP was defined as casesthat consisted primarily of pruritus, follicular hyper-keratosis, perifollicular inflammation/erythema, andpigment changes with a histopathologic correlationof an interface dermatitis. For pseudopelade, wedeparted somewhat from the NAHRS classificationand defined our cases as noninflammatory or verymild inflammatory cicatricial alopecias with no fol-licular hyperkeratosis. Lesions could be discrete asin classic pseudopelade (Brocq), but could also bepredominantly central with centrifugal spread. Weconsidered central centrifugal cicatricical alopecia avariant of pseudopelade. Histopathologic findingswere nonspecific with no vacuolar or interface

changes. FD or DC was defined as pustules or cystscausing scarring. Biopsy specimens showed pre-dominantly a neutrophilic infiltrative process.

RESULTSThere were a total of 112 new patients with pri-

mary cicatricial alopecias seen during a 5-year pe-riod. This represented 3.2% of the total number oftrichologic consultations seen at the UBC hair clinic.According to the NAHRS working classification forcicatricial alopecia, cases were categorized into lym-phocytic-mediated (CCLE, LPP, and pseudopelade),neutrophilic-mediated (FD and DC), and a mixedgroup (folliculitis keloidalis). The ratio of lympho-cytic versus neutrophilic or mixed was 4:1 (ie, 90 vs22 cases). The clinical characteristics of lymphocytic-and neutrophilic-mediated/mixed cicatricial alope-cias are summarized in Tables I and II, respectively.

Clinical characteristics of lymphocytic-mediated cicatricial alopecias (90 cases)

CCLE (38 cases). This represents 33.9% of thetotal number of cicatrizing cases seen in our cohort.There were 23 Caucasian (63.2%), 10 Oriental(26.3%), and 4 Middle Eastern/East Indian (10.5%)patients. The female to male ratio was 4.4:1 (31females and 7 males). The age at onset ranged from17 to 57 years, with a mean age of 35.6 years. Themajority of patients (78.9%) were seen within 1 yearof the onset of their clinical lesions. The 4 mostcommon symptoms were increased shedding/hairloss (100%), pruritus (65%), and scalp tenderness(26.2%). Salient clinical presentation consisted oferythematous plaques of alopecia, atrophy, telangi-ectasia, and follicular hyperkeratosis. Ulceration wasnoted in 13.2% of cases. The sites of involvementvaried from patient to patient, and there did notappear to be a predilection for a specific scalp site.The presence of hair tufting (polytrichia) was docu-mented in 4 cases.

The activity of the scalp disease was reflected bya positive hair pull and prominent follicular hyper-keratosis, particularly in the center of the plaque. Atotal of 11 patients (28.9%) with CCLE had concom-itant involvement of other body sites. Of 38 patients,8 (21%) were given the diagnosis of systemic lupuserythematosus. In all cases, histologic examinationwas consistent with the diagnosis of lupus erythem-atosus. Characteristic histologic findings includedfollicular vacuolar interface changes, and a perivas-cular and periadnexal dermal lymphoid infiltrate.Positive direct immunofluorescence was reported in20 of 32 cases (62.5%) in which IgG deposition wasfound along the dermoepidermal junction. Antinu-clear antibody test, performed in all cases, was pos-itive in 6 cases (15.8%).

26 Tan et al J AM ACAD DERMATOL

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Pseudopelade (27 cases). This category of cic-atrizing alopecia represents 24.1% of the total num-ber of cicatrizing cases seen in our cohort. Therewere 15 Caucasian (55.5%), 5 Oriental (18.5%), 4Middle East/Indian (14.8%), and 3 African American(11.2%) patients. The female to male ratio was 2.4:1(19 females and 8 males). There appeared to be 2main clinical patterns seen in our cohort. The firstgroup (n � 14) presented with multiple, asymptom-atic, 1- to 2-cm patches of cicatricial alopecic areasresembling classic “footprints in snow.” This presen-tation was more in keeping with the classic form ofpseudopelade of Brocq. In the second group, themajority affected were women between the ages of

40 to 60 years (n � 11) presenting with noninflam-matory centrifugal spread of cicatricial alopecia. Thislatter group was more in keeping with what theNAHRS classification refers to as central centrifugalcicatricial alopecia. Two patients had mild inflam-mation on the patch of cicatricial alopecia. The ac-tual extent of involvement was generally more ex-tensive than was appreciated by patients. The vertexand occipital scalp were frequently involved, al-though it may occur at any site. One man had pseu-dopelade affecting the beard. The average durationto diagnosis was 3.7 years (range: 7-10 years). Pseu-dopelade had been initially misdiagnosed as otherhair loss conditions such as androgenetic alopecia

Table I. Clinical spectrum of patients with lymphocytic cicatricial alopecias

Disease SexAge range

(y) Salient features Treatment

Chronic lupuserythematosus

31 Female7 Male

17-57 Erythematosus plaques withatrophy, telangiectasia,follicular hyperkeratosis.Activity of diseasepredominantly in thecenter of the plaque

Topical/intralesionalcorticosteroid: 38 (100%)

Oral corticosteroid: 10 (26.3%)Antimalarials: 19 (50%)Isotretinoin: 9 (23.7%)Methotrexate: 1 (2.6%)Topical minoxidil 5%: 5 (13.2%)Hair transplantation: 2 (5.3%)

Lichen planopilaris 16 Female9 Male

25-70 Usually solitaryerythematous plaque,complicated by ulceration,atrophy. Activity of diseasepredominantly in theperiphery of the lesion

Topical/intralesionalcorticosteroid: 25 (100%)

Oral corticosteroid: 4 (12.1%)Antimalarials: 19 (76%)Isotretinoin: 6 (24%)Topical minoxidil 5%: 6 (24%)Hair transplantation: 1 (4%)

Pseudopelade 19 Female8 Male

5-66 Multiple small oval to roundor reticulated patches onthe scalp

Topical/ intralesionalcorticosteroid: 27 (100%)

Antimalarials: 18 (66.7%)Topical minoxidil 5%: 9 (33.3%)

Table II. Clinical spectrum of patients with neutrophilic/mixed cicatricial alopecias

Disease SexAge range

(y) Salient features Treatment

Folliculitis decalvans 10 Male2 Female

17-45 Follicular papules,pustules, and plaques,tufting of hair;multiple; variable siteson the scalp, beard

Antibiotics: 12 (100%)Isotreinoin: 4 (33%)Topical minoxidil 5%: 3 (25%)

Dissecting folliculitis 6 Male 25-42 Coalescing deepnodules and plaques,abscesses, tufting ofhair; multiple; occiput,vertex

Antibiotics: 5 (83%)Isotretinoin: 4 (67%)Surgical reduction: 1 (17%)

Folliculitis keloidalis 3 Male 27-38 Keloidal plaques andnodules: occiput, napeof neck

Antibiotics: 1 (33%)Isotretinoin: 1 (33%)Surgical reduction: 2 (67%)

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(AGA; n � 6), alopecia areata (n � 3) (Fig 1), orother forms of cicatricial alopecias. In our study, 6patients (4 with LPP, 1 with CCLE, and 1 with FD)were initially given the diagnosis of pseudopelade.Subsequent biopsy specimens from fresh, active le-sions allowed us to reclassify them into their respec-tive entities. Histologically, perifollicular lympho-cytic infiltrate was reported with either a normalepidermis or an atrophic epidermis associated withan absence of rete ridges. There was an absence ofsebaceous glands and hair follicles. The end stage ofpseudopelade was characterized by marked fibrosisand lack of inflammatory infiltrate. Direct immuno-fluorescence was negative in all cases of what wedefined as pseudopelade.

LPP (25 cases). This category of cicatricial alo-pecia represented 22.3% of the total number of casesseen in our cohort. There were 19 Caucasian (76%),5 Middle Eastern/East Indian (20%), and 1 Chinese(4%) patient. The female to male ratio was 1.8:1 (16women and 9 men). Ages of the patients varied from25 to 70 years with a mean age of onset of 47.4 years.The majority of patients (80.2%) were seen within 1year of the onset of their clinical lesions. The 4 mostcommon symptoms of LPP were increased shed-ding/hair loss (100%), pruritus (90%), scale (80%),and scalp tenderness (72%). Lesions typical of lichenplanus were not found on the scalp. The majority ofthe lesions presented as scaly erythematous plaquesof cicatricial alopecia, which may be complicated byulceration and atrophy. Tufting of hairs was demon-strated in 5 patients (20%). Characteristic findingswere a positive pull for anagen hairs, perifollicularerythema, hyperkeratotic papules, and scaling inactive lesions (Fig 2). A total of 7 patients (28%) withLPP had extracranial lesions of lichen planus.

Concomitant AGA was diagnosed in 7 patientsand a woman had alopecia areata. Interestingly, awoman with a history of extensive alopecia areatadeveloped a cicatrizing process that spared anophiasic alopecic area affected by alopecia areata.

Three patients presented with a combination of ker-atosis pilaris on both arms and lichenoid lesions onother body sites, categorizing them as Graham-Little-Lasseur-Piccard syndrome. Nail changes, includepitting, trachyonychia, longitudinal ridging, and dor-sal pterygium, were documented in 10 patients(40%).

Histologic features consistent with LPP were fol-licular lymphocytic interface dermatitis, absence ofsebaceous epithelium, and perifollicular lamellar fi-brosis, with or without the presence of colloid bod-ies. In LPP, there was no increase in dermal mucin orsubcutaneous inflammation. Direct immunofluores-cence, revealing the deposition of globular IgM cy-toid bodies in follicular epithelium, was positive in 6of 25 cases (24%).

Treatment of lymphocytic cicatricial alope-cias at the UBC Hair Clinic. At the UBC hairclinic, patients were offered a variety of treatmentoptions for lymphocytic cicatricial alopecias.These included potent topical (class I or II), in-tralesional, or oral corticosteroids; antimalarials(chloroquine or hydroxychloroquine); andisotretinoin. In our study, the treatment responsewas based on photographic and both the physi-cians’ and patients’ global assessment. However,for certain patients it was difficult to evaluateefficacy as there were no defined NAHRS guide-lines at that time. If patients were asymptomatic,noninflammatory, or both at baseline, evaluationof efficacy was most problematic.

In all cases, intralesional and/or potent topicalcorticosteroids were first-line therapy. However, ste-roids were frequently combined with other systemicagents to achieve a remission in the majority ofcases. Intralesional corticosteroid (triamcinoloneacetonide, 10-20 mg/session) was administered on amonthly basis. Patients with active disease were re-viewed every 4 to 6 weeks. In the event of rapidprogression, oral corticosteroids were introduced tocurb the acute inflammation. Prednisone, started at a

Fig 1. Pseudopelade misdiagnosed as alopecia areata.Lack of follicular ostia may be difficult to appreciate insome cases.

Fig 2. Lichen planopilaris showing predominant diseaseactivity at periphery.

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dose of 1 mg/kg, was gradually tapered over 6 to 8weeks. Intermittent short courses of prednisonewere frequently required to control acute flares ofboth lupus erythematosus and LPP.

In our center, antimalarials and isotretinoin weresecond-line therapies because of their potential sideeffects. Hydroxychloroquine (400 mg/d) was fre-quently added in many cases (n � 54; 48.2%). In ourexperience, antimalarials were required for a mini-mum duration of 9 months before they could betapered. On the average, antimalarials were givenfor an average duration of 15 months (range: 9-24months) before they could be discontinued. Isotreti-noin, at a dose of 1 mg/kg, was given to 15 patients.Among them, 10 had failed to achieve a good clin-ical response to antimalarials, corticosteroids, orboth. Ten experienced resolution of inflammationafter approximately 4 months of therapy. The aver-age duration of isotretinoin therapy was 8 months(range: 6-12 months). One patient with refractoryCCLE was prescribed methotrexate with minimalbenefit. Of 20 patients who were prescribed minoxi-dil 5% lotion, 12 thought that there was a mild tomoderate degree of hair regrowth. Three patientsunderwent hair transplantation after disease remis-sion was attained for at least 2 years.

Clinical characteristics of neutrophilic/mixedcicatricial alopecias (22 cases)

There were 13 cases of FD (10.7%), 6 cases ofDC (4.5%), and 3 cases of folliculitis keloidalis(1.8%).

FD. FD was diagnosed in 10 men and 3 women(male to female ratio of 3.3:1). There were 8 MiddleEast/East Indian, 3 Caucasian, and 2 African Ameri-can patients. Ages of patients ranged from 21 to 45years with an average of 33 years. There was nopredilection for any particular site. Variable acne-iform morphologic presentations including multiplefollicular papules, pustules, and plaques were ob-served. These lesions tended to be multiple, painful,

and were distributed over the entire scalp (Fig 3).Healing of these lesions left multiple 0.5- to 1-cmpink-white scars that sometimes resembled pseu-dopelade. Concomitant involvement of the beardwas observed in 2 men. Neutrophilic cicatrizing alo-pecias tended to wax and wane, and run a pro-tracted course. Tufting of hairs/polytrichia wasnoted in most cases of FD. Swab cultures werepositive for Staphylococcus aureus in 10 of 13 cases.

DC. A total of 6 men (3 Caucasian, 1 Indian, and2 African American) presented with inflammatorytender plaques and nodules on the scalp that wereclinically consistent with DC (perifolliculitis capitisabscessens et suffodiens). None of the patients hadassociated acne conglobata and hidradenitis suppu-rativa. Two African American men presented withfibrotic, hypertrophic plaques and nodules on theocciput and nape of neck. Tufting of hairs/poly-trichia was also observed in DC (Fig 4).

Pathology of FD and DC. Similar histologicfeatures were reported in these 2 subgroups. Salienthistologic features included inflammatory accumu-lation of mostly neutrophils, with a component ofboth lymphocytes and plasma cells at varyingdepths of the biopsy specimens. In quiescent stages,the histologic picture of dermal and subcutaneousfibrosis was reported.

Treatment of neutrophilic cicatricial alope-cias at the UBC Hair Clinic. Both FD and DC runa variable clinical course. Broad-spectrum antibiot-ics were first-line therapy in all patients. Frequently,intralesional/potent corticosteroids were added tocurb the acute inflammation. Broad-spectrum anti-biotics (tetracycline, minocycline, cloxacillin, eryth-romycin, or broad-spectrum cephalosporin) werestarted at the dose that was frequently used to treatacne vulgaris. Gradual tapering was tailored accord-ing to the individual clinical response. The averageduration of antibiotic therapy was 10 months (range:6-24 months). However, a high relapse rate wasobserved in 20 cases after stopping antibiotics. In 4

Fig 3. Active stage of folliculitis decalvans showing mul-tiple pustules.

Fig 4. Dissecting folliculitis demonstrating polytrichia/tufted folliculitis.

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individuals who did not respond to single antibiotictherapy, a combination of clindamycin and rifampi-cin, both at 300 mg twice daily for 12 weeks, wasattempted. However, the disease relapsed within 6months of stopping the combination. Isotretinoinwas started in 9 patients with refractory disease. Adose of 1 mg/kg was started and maintained for atleast 6 months before the dose was tapered. Theaverage duration of treatment was 9 months (range:6-18 months).

Folliculitis keloidalis. A total of 3 patients pre-sented with hypertrophic plaques and nodules onthe occipital scalp and the nape of their neck. Theyhad surgical excision of the hypertrophic lesionswith good cosmetic outcome. There was no recur-rence of keloidal scar after excision.

DISCUSSIONThere is a paucity of data on cicatricial alopecia

worldwide.1,2 The inflammatory process predomi-nantly occurs around the permanent portion of thehair follicle (stem cells of the bulge area and theinfundibulum), hence resulting in the irreversibilityof alopecia.2,3 Our study aimed to review the epide-miologic profile, clinical characteristics, and treat-ment outcome of patients with primary cicatricialalopecia seen in a mixed ethnic community. At theUBC hair clinic, we reported a greater proportion(4:1) of lymphocytic cicatricial alopecias versus neu-trophilic cicatricial alopecias, which is in agreementwith data from Whiting.2

Lymphocytic cicatricial alopeciasIn order of frequency, CCLE and LPP followed by

pseudopelade were the 3 commonly occurring cic-atricial alopecias. In accordance with previous stud-ies, we observed a female predominance of lympho-cytic cicatricial alopecias.2-4 In our series, both LPPand CCLE affected predominately middle-agedadults, although LPP appears to have a later onset inlife. In lupus erythematosus, the activity of the dis-ease is primarily focused in the center of the plaque,whereas in LPP the activity predominately occurredat the periphery.3 Scalp tenderness and prurituswere more pronounced in LPP.3,5 A positive hair pulltest assisted in determining the activity of disease.3,5

The diagnostic challenge is in burnt-out stages ofLPP and CCLE when it is often clinically and histo-logically impossible to differentiate from other formsof cicatricial alopecia.2,3 Repeated scalp biopsy spec-imens from new, active lesion may be necessary tomake a definitive diagnosis. CCLE of the scalp mayprecede, coexist, or follow other skin lesions.6 In anearlier study by Wilson et al,4 34% of patients withCCLE had scalp involvement and this may serve as amarker of chronicity and poor response to treat-

ment. In a smaller study by Callen,7 41% of patientswith CCLE had cicatricial alopecia. In our series,approximately 25% of the patients with scalp CCLEhad extracranial manifestations of CCLE. All of ourpatients with extracranial involvement required sys-temic therapy.

Classic presentation of lichen planus is not usu-ally demonstrated on the scalp.8-13 On the scalp, theclassic clinical features include the presence of fol-licular hyperkeratotic papules, perifollicular ery-thema, and scaling at the periphery of the cicatricialprocess. Onychodystrophy was recorded in 40% ofLPP and we thought that associated nail changesmight not be that uncommon in LPP.

The term “pseudopelade” is confusing and de-notes different meanings to different authors.2,3,14-16

Sperling et al14 has attempted to redefine pseudopel-ade of Brocq, follicular degeneration syndrome, andFD as subsets of central centrifugal scarring alope-cia. In the recent NAHRS classification on cicatricialalopecia, classic pseudopelade of Brocq and centralcentrifugal alopecia belong to 2 distinct entities.1 Inour study, we defined pseudopelade as both theclassic reticulated footprints in snow pattern and thecentral variant. Pseudopelade may not be an uncom-mon occurrence in children and it is important tobear in mind this diagnosis whenever one encoun-ters a child with patchy alopecia.17 However, themajority of pseudopelade behaves in an insidiousmanner and is rarely inflammatory.1,15

In our experience, pseudopelade is a great mim-icker of many hair-loss conditions.3,18 The centralcentrifugal form of pseudopelade can mimic AGAand we have seen at least 2 male patients inappro-priately put on treatment for AGA by their referringphysicians. The classic type of Brocq can mimicalopecia areata. In our series of patients, pseudopel-ade was misdiagnosed as alopecia areata, AGA, andother forms of scarring alopecias. Its noninflamma-tory nature frequently prevents early diagnosis. Ed-ucation of physicians in recognizing cicatrizing alo-pecias is pertinent to facilitate early diagnosis andavoidance of inappropriate treatment such as finas-teride.

The main treatment goal in cicatrizing alopecias isto arrest the cicatrization process, decrease follicularinflammation, and halt further irreversible destruc-tion. However, treatment of all types of primarycicatrizing alopecias can be difficult, as there are noconsistent markers to chart treatment progress.Treatment approaches are based on both the sever-ity of disease and the patient’s tolerance of treat-ment.

At the UBC Hair Clinic, for lymphocytic cicatriz-ing alopecias, intralesional and potent topical corti-

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costeroids are prescribed routinely in all active casesto reduce the inflammatory component while mini-mizing systemic side effects. Intermittent shortcourses of oral corticosteroids are helpful in control-ling the acute inflammatory stages of LPP and lupuserythematosus. Systemic agents are reserved as sec-ond-line therapy. However, in the majority of cases(n � 54; 58.1%), a systemic agent was required tohalt the inflammatory process. Antimalarials andisotretinoin have been used previously with variablesuccess in both LPP and CCLE.2,3,19-21 We also thinkthat patients with severe disease do benefit fromseveral months (at least 6 months) of treatment atfull dose. A gradual tapering of antimalarials orisotretinoin may offer a longer disease remission.The role of topical minoxidil 5% in cicatrizing alo-pecias is controversial. Of 23 patients who wereprescribed topical minoxidil 5% solution, 12 derivedmild to moderate hair regrowth after 6 months. Theexact mechanism of minoxidil for cicatricial alopeciais unknown. It is possible that many of the femalepatients with pseudopelade may have had concom-itant AGA and, as a result, benefited from the overallincrease in hair density from topical minoxidil solu-tion. We agree with other authors that a trial oftopical minoxidil 5% solution for a year may providebenefit in scalp coverage for certain cases of cicatri-cial alopecia.2 Hair transplantation may be a feasibletreatment option when the disease has been quies-cent for at least 2 years.3,5

Neutrophilic/mixed cicatricial alopeciasThe pathogenesis of neutrophil cicatrizing alope-

cia in unknown. S aureus is usually cultured frompustules but it is unclear whether this is a primary orsecondary process. The possible role of bacterialsuperantigens or a defect in cell-mediated immunityhas been postulated in neutrophilic cicatrizing alo-pecias.2,3 A male preponderance has been observedin all subtypes of neutrophilic cicatrizing alope-cias.2,22-24 Sites of occurrence may vary, but we ob-served preferential involvement of the posterior halfof the scalp. In addition to the scalp, other hairbearing areas may be affected. This is consistentwith previous reports.2 We believe that FD, DC, andfolliculitis keloidalis represent a different spectrumof the same disease. Underlying differences in fol-licular anatomy or host response may be responsiblefor the different clinical manifestations. Burnt-out orquiescent stages of neutrophilic cicatrizing alopeciashave engendered some confusion with pseudopel-ade.

DC or perifollicular capitis abscedens et suffodi-ens of Hoffman is another inflammatory condition ofthe scalp that can lead to cicatrization. It usually

presents with deep, inflammatory nodules primarilyover the occipital scalp that may coalesce to form aboggy scalp. Sinus tracts may form and dischargepurulent materials. Folliculitis keloidalis, typicallydescribed in African American patients, presentswith follicular papules, plaques, and areas of fibrosison the posterior scalp. In our patients distinctivetufts of multiple hairs were seen emerging fromsingle follicular openings within the scarred areas.We tend to agree with the explanation that hairtufting is a secondary phenomenon that may occurin several different inflammatory disorders of thescalp, rather than a diagnostic feature of a specificdisease.25 In our study, tufting of hair was noted inall forms of cicatrizing alopecia.

Treatment of neutrophilic cicatrizing alopecias isfrequently dissatisfying. A common denominatorhas been a high relapse rate after stopping treat-ment. The first-line treatment consists of combina-tion therapy with systemic antibiotics and intrale-sional corticosteroids. The choice of antibioticsincludes tetracycline, minocycline, cloxacillin, eryth-romycin, or broad-spectrum cephalosporin. We be-lieve that antibiotic therapy, at doses equivalent tothose used for acne vulgaris, is often required formany months.2,26 Isotretinoin has been shown to besuccessful in previous reports.27,28 In our experi-ence, isotreinoin was able to induce disease remis-sion temporarily in all treated cases. We agree withBjellerup and Wallengren28 that isotretinoin is likelyto maintain a longer disease-free interval comparedwith oral antibiotics. For discrete and limited lesions,surgical excision of the scarred areas is an accept-able form of treatment.

CONCLUSIONCicatricial alopecias are trichologic emergencies,

as the resultant alopecia is often irreversible. Aggres-sive management may aid in follicular rescue. Anaccurate diagnosis is achieved through a careful clin-icopathologic evaluation. We suggest that a scalpbiopsy is mandatory in all cases and multiple biop-sies may sometimes be necessary to make a defini-tive diagnosis. The high variability in the clinicalcourse of primary cicatrizing alopecias makes diag-nosis and management challenging for dermatolo-gists. An aggressive multiple modality therapeuticapproach is often necessary to prevent further fol-licular destruction.

REFERENCES1. Olsen E, Bergfeld W, Cotsarelis G, Price V, Shapiro J, Sinclair R, et

al. Summary of North American hair research society (NAHRS)sponsored workshop on cicatricial alopecia. J Am Acad Derma-tol 2003;48:103-10.

2. Whiting DA. Cicatrizing alopecia: clinico-pathological findingsand treatment. Clin Dermatol 2001;19:211-25.

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