Pseudopelade of Brocq(1).pdf

Dermatologic Therapy, Vol. 21, 2008, 257–263

DERMATOLOGIC THERAPY

ISSN 1396-0296

Blackwell Publishing Inc

Pseudopelade of Brocq

BDULLATEEF

A. A

LZOLIBANI

, H

OON

ANG

, N

INA

TBERG

ERRY

HAPIRO

Department of Dermatology and Skin Science, University of British Columbia,

Vancouver, British Columbia, Canada

Pseudopelade of Brocq (PPB) is a rare, idiopathic, slowly progressive hair disorder,

resulting in cicatricial alopecia. It typically presents in Caucasian adult patients as small, smooth,

ﬂesh-toned and slightly depressed alopecic patches with irregular outlines. It primarily involves the

parietal and vertex portions of the scalp with a chronic prolonged course. Controversial opinions still

exist as to whether PPB is a single entity or an end stage of several cicatricial alopecic disorders. A

practical approach to diagnosis of PPB and therapeutic update are discussed in this review.

KEYWORDS:

cicatricial alopecia, primary lymphocytic, pseudopelade of Brocq, review

Background

unsuccessful to date (4). Amato et al. (21,22)

studied the histopathological and immunopatho-

logical features of 36 patients diagnosed with PPB

according to the clinical criteria of Braun-Falco

et al. (8) They identiﬁed that 69% of their patients

were secondary to LPP or DLE, and this percentage

is higher than previously reported by Braun-Falco

et al. (8) who in their case series of 26 patients,

reported 33% of cases showing histological evidence

of DLE or LPP. A deﬁnitive pathological diagnosis

and reliable histopathological features distinguishing

LPP from pseudopelade could not be made by

Nayar et al. in their study of 10 patients with

clinical evidence of LPP or pseudopelade (16).

Similarly, Annessi et al. have reported that 16 out

of 25 of the biopsy specimens that were clinically

characterized as pseudopelade showed histological

ﬁndings similar to those seen in late-stage LPP

(26). Moretti et al. (27) studied 12 patients with

the clinical diagnosis of PPB, according to Braun-

Falco’s (8) criteria and identiﬁed 5 of 12 patients

were secondary to LPP or DLE by means of histology

and direct immunoﬂuorescence (DIF). By using

immunohistochemistry, the phenotypic pattern of

dermal inﬁltration can allow further differentiation

of secondary (richer in lymphocytes) from idio-

pathic PPB (richer in macrophages, mast cells, and

ﬁbroblast) (27). These ﬁndings support the existence

of a primary, idiopathic form of PPB. The NAHRS

consensus group’s deﬁned classic PPB as “clinically

discrete, smooth, ﬂesh-toned areas of alopecia

without follicular hyperkeratosis or perifollicular

inﬂammation” (28).

is used

in the literature to describe both PPB and other

forms of cicatricial alopecia that simulate it.

The concept of PPB is highly controversial.

More than 100 years later, the debate continues

as to whether PPB is idiopathic or secondary to

another condition (4). Many authors consider PPB

as a distinct clinicopathological entity (5–12). Other

authors believe that PPB is the common ﬁnal

stage or clinical variant of several different forms

of scarring alopecias mainly lichen planopilaris

(LPP) and discoid lupus erythematosus (DLE) (13–

23). Abandoning the use of the term

pseudopelade

had been recommended by several authors

(10,18,24,25). In this review, we will restrict our

use of term pseudopelade of Brocq to the pattern

of hair loss described by Brocq et al. (3) Attempts

to deﬁne PPB clinicopathologically have been

Address correspondence and reprint requests to: Abdullateef A.

Alzolibani, MD, Clinical and Research Fellow, Department of

Dermatology and Skin Science, University of British Columbia,

835 West 10th Avenue, Vancouver, BC, Canada V5Z 4E8, or

email: azolibani@yahoo.com.

257

& J

ABSTRACT:

Pseudopelade of Brocq (PPB) is an idiopathic,

chronic, slowly progressive primary lymphocytic

cicatricial alopecia. “Pseudo-pelade” was ﬁrst

described by Neumann in 1869, but named by

Brocq in 1888 for its similarity to hair loss of alope-

cia areata (1,2). In 1905, Brocq et al. described in

detail the clinical features of this disorder as a

unique entity (3). The term

Alzolibani et al.

As in other primacy lymphocytic alopecias,

the exact pathogenesis of PPB is still unknown.

Different theories exist about the scarring process.

The inﬂammatory cell inﬁltrate in PPB is particu-

larly located around the follicular bulge in which

the pluripotent follicular stem cells are located.

Damage of the follicular stem cells in the bulge–

isthmus area may explain the scarring nature of

PPB (12,17). Hypotheses have been implicated in

the pathogenesis of PPB other than idiopathic

acquired autoimmunity (16,29), such as

Borrelia

which has been detected in some

cases of PPB (30,31).

Clinical features

FIG. 1. Pseudopelade of Brocq with irregular patches of

scarring alopecia.

The epidemiology of primary cicatricial alopecias

in the general population is unknown (32). The

prevalence has been reported to range from 3.2%

to 7.3% in two retrospective studies within a hair

clinic setting. (33,34). Pseudopelade accounted

for 10%, 24.1%, and 40.6% of cases of cicatricial

alopecia in three retrospective studies from dif-

ferent centers (33–35). Differences in patient

demographics, referral pattern, and criteria for

clinicopathologic diagnosis of pseudopelade

probably account for this wide range (33,36). The

reports of the PPB in non-Caucasian patients are

still lacking (4). PPB primarily affects females

(5,11,12,15,22,33,34,37). Onset is usually during

adulthood between 30 and 50 years of age (3,7,12,18),

although the condition may occur in childhood

(36,38–40). A familial form of disease has also

been reported, and it was described in a mother

and son in one report (40), and in two brothers in

another report (39).

Pseudopelade of Brocq is a chronic, insidious

form of primary lymphocytic cicatricial alopecia.

It is characterized by multiple, small, discrete,

asymmetrical, smooth, soft, ﬂesh-toned or white

alopecic patches with little, if any, clinical

inﬂammation (3,4,12,34). Three patterns of pseudo-

pelade including scattered, large plaques, and a

combination of both morphologies have been

described (3). The parietal and vertex of the scalp

are primarily involved (4,12,41). (FIGS. 1 and 2)

Initial lesions typically present as small, round to

oval plaques and are asymptomatic. Mild pruritus

and diminished lesional sensation may be present

(4). In addition, mild perifollicular erythema

may be detected, but in general there is little or

no inﬂammation (8,11,17,18,33). Rarely, slight

perifollicular scale may be present in early stages

(4,18). In light-skinned individuals, conﬂuent

FIG. 2. Close-up view of the lesions in Fig. 1.

plaques of alopecia with a shiny, hypopigmented,

porcelain-white appearance are typically seen

(3,4,6,7). Infrequently, diffuse or pale rose coloration

can be seen (4). The lesion is often slightly

depressed (4,18). The classic description of “foot-

prints in the snow” refers to lesional depression

caused by moderate dermal atrophy and by a

reticulated pattern with small irregular patches

(FIG. 3). In fact, many cases lack this atrophic

feature and atrophy was originally omitted as a

typical feature (4–6,18,28). Evolution of the lesions

may result in confetti-like distribution; reticulate

pattern; or coalescence into larger alopecic

patches with polycyclic borders (4). The hair

shafts at the edge of an active alopecic patch are

easily extractable and may be twisted (34). Typical

of many forms of primary scarring alopecia,

scattered isolated and grouped hairs may remain

in the patches of alopecia and may appear curly

due to follicular distortion (12,17,42) (FIG. 4).

258

burgdorferi

Pseudopelade of Brocq

FIG. 3. Pseudopelade of Brocq scarring alopecic patches

with classic “footprints in the snow” appearance.

(CCCA) (4,12,33,34). The lesions of PPB are ﬂesh

toned or hypopigmented, sometimes depressed,

irregular alopecic patches without follicular

openings, as opposed to round to oval nonscarred

patches of alopecia areata (18,19). Typical excla-

mation mark hairs and associated nail ﬁndings

such as pitting will help in the diagnosis of alopecia

areata. Asymptomatic scarring alopecia without

inﬂammation or scales is the initial clue for the

diagnosis of PPB. Perifollicular scale is more sug-

gestive of LPP or DLE. In active LPP, acuminate

follicular lesions with perifollicular keratotic plugs

are seen at the margins of alopecic patches.

Inﬂammation is less obvious in PPB Typical

lesions of lichen planus on glabrous skin or

mucous membranes can be found in up to 50% of

patients with LPP (46). In some cases, PBB cannot

clearly be differentiated from LPP. The diagnosis

of lupus erythematosus may be easy to make in

an active early lesion especially in the presence of

extracranial involvement, but advanced cases

may not be so distinctive (34). Dyspigmentation

and subcutaneous atrophy associated with “burnt

out” DLE are helpful signs (17). Immunoﬂuorescence

is useful in diagnosing scarring DLE; a positive

lupus band will be found in about 76% of

patients, with IgG, IgM, or complement deposits

(22,34). Antinuclear antibody test is helpful, if

DLE is being considered as differential diagnosis.

An expanding area of symmetric alopecia patch

and central scalp location are characteristic of

CCCA, which is clinically quite distinct from the

classic form of PPB (12). Irregularly outlined and

typically atrophic alopecic plaques are seen in

PPB, sometime with coexisting lenticular disease

which is not seen in patients with CCCA (4).

Exclusive crown or vertex involvement may actually

represent cases of “burnt-out” CCCA (18,19,23).

PPB often worsens in “spurts” with periods of

progression followed by “dormant” periods which

is different from the slow but steady progressive

course of CCCA (18,19,23). Although the histo-

pathological features of CCCA may be similar to

those seen in classic PPB, a good clinical history

and scalp examination will be helpful in differen-

tiating the two conditions (12). Insidious progres-

sive alopecia caused by sarcoidosis should also be

excluded (47). Sarcoidosis may present with

ﬂesh-colored to telangiectatic yellow, indurated

alopecic plaques and nodules. The areas of localized

or diffuse scarring alopecia may demonstrate an

inﬁltrated border (37,47). Occasionally, PPB can

mimic male pattern hair loss (33,36). Other rare

entities including morphea, tinea capitis, and

secondary syphilis need to be excluded. Morphea

FIG. 4. Noninﬂammatory scarring alopecia of pseudo-

pelade of Brocq. Note absence of scale and inﬂammation, and

presence of isolated and grouped hairs in the patches of

scarring alopecia.

Although the scalp is the classic site of PPB, con-

comitant beard involvement has been reported

(3,43). In addition, eyebrow loss (madarosis) with

pseudopelade has been reported (44,45). The

course of disease is variable. The majority of the

cases undergo a prolonged and slowly progressive

course over many years with development of

small alopecic patches that ultimately coalesce

together producing larger irregular areas of scarring

hair loss (3,43). Occasionally, rapidly progressive

cases have been reported (3,7).

Differential diagnosis

Pseudopelade of Brocq may mimic alopecia areata,

LPP, DLE, and central centrifugal cicatricial alopecia

259

Alzolibani et al.

may present with solitary or a few patches of

alopecia with a typical yellow to white color (37).

Scalp biopsy and direct immunoﬂuorescence will

help in the ﬁnal diagnosis.

clinicopathologic and immunopathologic study

of 136 cases of cicatricial alopecia by Trachsler

and Trueb, it was found that an accurate diagnosis

could be made in 97% of diagnosed cases on the

basis of histopathology alone and independently

of DIF ﬁnding (35). Similar results were found in

another study of 108 patients with cicatricial

alopecia done by Stavrianeas et al. in which histo-

pathology alone allowed accurate diagnosis in

92% of the cases (48). The authors suggest that

DIF should not be performed on a routine basis

but using it only in histopathologically inconclusive

cases of cicatricial alopecia (35,48). On the contrary,

Mirmirani et al. in their prospective and blinded

histopathological evaluation of 12 biopsy specimens

of clinically typical primary cicatricial alopecia

showed the difﬁculty of using histological criteria

to distinguish between primary cicatricial alopecias

in the lymphocytic group (LPP, frontal ﬁborsing

alopecia, PPB, and CCCA) (49). An interesting point

in this study was the evidence of the disease in 8

of 12 biopsy samples of clinically unaffected scalp

(49). Fabbri et al. found in their retrospective

study of 36 patients with scarring alopecia of the

scalp secondary to DLE, the histopathology alone

allowed a correct diagnosis in 68.5% of the cases

(50). The remaining cases (31.5%) were diagnosed

by immunopathologic ﬁndings; this reﬂects the

signiﬁcant value of immunopathologic ﬁndings in

making the correct diagnosis.

In advanced disease, elastin stains are important

in differentiating PPB from LPP and discoid lupus

erytheromatosus. Using an acid-alcohol orcein

stain, dense elastic tissues are seen around the

lower part of the follicle, unlike other scarring

alopecia in which the scar tissue consists of

collagen devoid of elastin (51,52). Although less

speciﬁc, ﬂuorescent microscopic analysis of

routinely stained sections can be used for rapid

and simple assessment of elastin pattern in

histological evaluation of cicatricial alopecias (53).

In an atrophic process, the elastic ﬁber network

could be selectively spared, whereas in a scarring

one it is destroyed. There is remarkable preservation

of elastic ﬁbers in PPB. The appearance of thick

elastic ﬁbers may be secondary to dermal collagen

contraction resulting in recoiled elastic ﬁbers (37).

In this review, the authors had suggested to

reclassify PPB as a permanent but atrophic rather

than scarring alopecia (12). As mentioned above,

the elastic ﬁber stain and immunohistochemistry

of dermal inﬁltration support the existence of

PPB as unique entity. However, until detection of

speciﬁc immunophenotyping and unique mole-

cular markers of PPB, the subject of ongoing debate

Pathology

A scalp biopsy is the ﬁrst step in the management

of PPB and other cicatricial alopecias. The correct

biopsy technique is crucial for the diagnosis. Two

4-mm deep punch scalp biopsies oriented along

the direction of the hair follicle for both routine

and DIF histopathology are taken from the border

of an early, clinically active alopecic patch. Scarred,

hair bearing and nearby clinically normal perilesional

areas should be included in the biopsies. One

punch biopsy is submitted for horizontal sectioning

and the other is divided in half and submitted for

both immunoﬂuorescence and longitudinal

sectioning (36,41). Bacterial and fungal infections

should be excluded in all cases of cicatricial

alopecia (34).

No pathognomonic pathologic features of PPB

have been described (25,37). Routine histological

examination of the classic PPB usually shows

nonspeciﬁc features and the expected ﬁndings are

those of a burned-out scarring alopecia (16,18,37).

Some authors believe that PPB is a diagnosis of

exclusion (18,19,23). Classic PPB shows no interface

changes in contrast to primarily vacuolar in DLE

and primarily lichenoid in LPP (11,12). Early lesions

are characterized by a sparse to moderate perifol-

licular and perivascular lymphocytic inﬁltrates

without associated interface alteration. The

lymphocytic inﬂammation is found primarily at

the level of the follicular infundibulum (8,11,12,34).

The sebaceous glands are reduced or absent and

there is atrophy of follicular epithelium (8,12,34).

As the alopecia develops, the infundibular epithelium

becomes atrophic (11). Characteristic concentric

lamellar ﬁbroplasia in an onion skin–like pattern

is present and are more prominent in advance

stage (11,12,34). End-stage disease is marked by

complete loss of the folliculo-sebaceous unit with

the formation follicular longitudinal ﬁbrous tracts

that extend into the subcutis. Residual arrector

pili muscles, hair-shaft granulomas and sparse

lymphohistiocytic inﬁltrate may be observed

(8,11,12,26).

Direct immunoﬂuorescence is negative or occa-

sionally reveal ﬁnely granular IgM deposition along

the follicular infundibular basement membrane

distinct from the typical pattern seen in DLE

and LPP (8,10–12,17,26,29). In a retrospective

260

Pseudopelade of Brocq

about the delineation of PPB from other end-stage

LPP or DLE will continue. With all types of investi-

gations, the minority of unclassiﬁed, nonspeciﬁc

cases of cicatricial alopecia still exist (28,33,35,54),

reﬂecting the present limited understanding of

those disorders. Important factors that affect the

accurate diagnosis of cicatricial alopecia include

optimum biopsy site, correct biopsy technique,

and an experienced pathologist with a special

interest in scalp pathology. Sometimes, repetitive

scalp biopsies from new active lesions may make

a deﬁnitive diagnosis, owing to a highly variable

clinical course in some cases of primary scarring

alopecia.

scalp oil

(ﬂuocinolone acetonide) applied once at night

and Clobex shampoo (clobetasol propionate)

once daily in the morning for 15 min will help in

alleviating the symptoms and removing scales (A.A.

and J.S., personal observation). Topical tacrolimus

0.1% ointment or in Cetaphil lotion twice daily

can be added. A trial of topical minoxidil 5%

solution or foam twice daily for 1 year may provide

beneﬁts in some cases of cicatricial alopecia

(33,34). Some patients can be maintained with

topical therapy alone. In cases of failure to local

therapy after an 8- to 12-week trial, or more

extensive lesions (more than 10% scalp involve-

ment) or rapidly aggressive cases, hydroxychloro-

quine (200 mg twice daily) alone or in combination

with oral prednisone is indicated (4,33,34,36,38,

40,41,55,56). Oral prednisone at a dose of 0.5 mg/

kg and tapering course over 2 months is used as

bridge therapy to induce remission while the

antimalarial starts its effect. Sometime, intermittent

short courses of prednisone are needed to control

ﬂare-ups of the disease. Hydroxychloroquine is

antilymphocytic (56) and is considered as the ﬁrst

line of systemic therapy. A baseline ophthalmologic

examination including a retinal examination,

complete blood count, and liver function tests are

required before starting hydroxychloroquine

therapy. The blood tests are usually repeated

every 3 months and the ophthalmologic examina-

tion is repeated every 12 months. The patients are

assessed every 3 months. If there is good response

to hydroxychloroquine, which is usually evident

during the ﬁrst 3–6 months, the same dose can be

continued for a total of 9–12 months. Thereafter,

the dose of hydroxychloroquine is gradually tapered

to 200 mg once daily for 3–4 months then every

other day for another 3–4 months. Most patients

require 1–2 years of antimalarial therapy. In cases

of failed oral antimalarial treatment of 6 months,

other therapeutics options can be considered,

including isotretinoin (4,33,41) and mycophenolate

mofetil (4,33,56).

The use of isotretinoin (at a dose of 1 mg/kg/day

for 6–12 months) in the treatment of PPB had

been reported, but in our experience is not effective

against PPB. Mycophenolate mofetil is an immu-

nomodulating drug that speciﬁcally inhibits the

proliferation of activated T lymphocytes (56).

Before starting mycophenolate mofetil therapy,

baseline laboratory studies are done including a

complete blood count, liver function tests and

negative pregnancy test (also repeated 1 week after

starting therapy). Double reliable contraception

Therapeutic management

The chronic course, permanent destruction of the

hair follicles and treatment difﬁculty of the disease

must be explained to the patient. The activity of

the disease is determined by a positive pull test

result, extension of the alopecic patches, and

occasionally symptomatic lesions. The goal of

treatment is to induce clinical remission and

prevent further progression. Unfortunately, even

when treatment relieves the symptoms and signs,

the progression of hair loss may continue. There is

difﬁculty in monitoring the therapeutic efﬁcacy of

the treatment of PPB because of mostly asymp-

tomatic lesions with no overt clinical signs (4,33).

However, careful measurements and meticulous

close-up photography of the lesions to assess

the extension of scalp involvement may help.

Sometimes, a follow-up biopsy is used to assess the

treatment response. The treatment recommenda-

tions in this review reﬂect our practice experience

in the Department of Dermatology and Skin

Science at the University of British Columbia.

Selection of treatment option is more challenging

than expected. Current treatment options are

limited and may not change the actual outcome

of the disease. The therapeutic approach is almost

similar to LPP. The choice of treatment depends

mainly on the activity, extent of the disease and

patient’s tolerance of treatment. For active localized

lesions (less than 10% scalp involvement), a

combination of topical (4,33,34,36,41,55,56) and

intralesional corticosteroid (4,33,34,36,54,55) is

initiated. Intralesional triamcinolone acetonide

injections with a concentration of 10 mg/mL at a

dose of 2 mL monthly, and twice daily application

of high potency, class I or II topical corticoster-

oids(lotion/cream/ointment) is applied. Dermasone

lotion or cream (clobetasol 17 – propionate) applied

261

twice daily is recommended as ﬁrst-line topical

corticosteroids. Derma-Smoothe/FS

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