The President 2010-12

TTS President 2010

 

 

 

 

 

 

the Trichological Society

 

PSEUDO-PELADE OF BROCQ

Pseudo-pelade of Brocq (false Alopecia Areata) is a rare disease which initially resembles commencing alopecia areata from which is name derives.

The scalp exhibits one or more small bald patches which are smooth and atrophic. Many more such patches may appear.


Sites: any scalp location but usually the vertices and parietal regions.

The disease has given rise to the phrase 'footsteps in the snow'

 

© The Trichological Society

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PSEUDO-PELADE OF BROCQ    Overview by Inga Zemite MD MTTS

Contents

  • Classification of Primary Cicatricial Alopecias
  • Introduction on PB
  • History and Epidemiology
  • Pathogenesis
  • Clinical features
  • Laboratory investigations and Pathology
  • Differential diagnosis
  • Treatment
  • References

1. Classification of Primary Cictricial Alopecias.

The term cicatricial alopecia encompasses a diverse group of disorders characterized by permanent destruction of the hair follicle and irreversible hair loss.
In 2001 the North American Hair Research Society (NAHRS) developed a working classification of PCA based on the type of inflammatory cells that destroy the hair follicle during the active stage of the disease. The inflammation may predominantly involve lymphocytes or neutrophils, and therefore primary cicatricial alopecias as classified by NAHRS are “lymphocytic” or “neutrophilic”.


Inflammatory infiltrate

Diagnosis

Lymphocytic

Chronic cutaneous lupus erythematosus
Lichen planopilaris (LPP)
Classic LPP
Frontal fibrosing alopecia
Graham Little syndrome
Classic pseudopelade (Brocq)
Central centrifugal cicatricial alopecia
Alopecia mucinosa
Keratosis follicularis spinulosa decalvans

Neutrophilic

Folliculitis decalvans
Dissecting cellulitis/folliculitis (perifolliculitis abscedens et suffodiens)

Mixed

Folliculitis (acne) keloidalis
Folliculitis (acne) necrotica
Erosive pustular dermatosis

Nonspecific

Can be end – stage of all conditions

Table 1.1. The North American Hair Research Society Classification of Primary Cicatricial Alopecias.

2. Introduction on PB
Pseudopelade of Brocq (PB) comes under the category of lymphocytic cicatricial alopecias. This entity is characterized by permanent destruction of the hair follicle.
In 1888, Brocq first used the term pseudopelade to describe a form of scarring alopecia that clinically is similar to alopecia areata. After a study of the disorder, in 1905, Brocq, Lenglet and Ayrignac concluded that pseudopelade is a unique classifiable entity.
Some researchers maintain view that PB is very distinct clinico-pathologically, while others still consider it as a variant of certain other primary alopecias or the end stage of other conditions, for example DLE, LLP and other forms af cicatricial alopecias. PB can be considered as pattern of cicatricial alopecia. If a definitive diagnosis of DLE, LLP, or another condition can be made based on clinical, histological, or immunofluorescent features, then the term pseudopelade of Brocq cannot be used. A primary form of traditional pseudopelade may exist.
It is not understood how or why pseudopelade of Brocq occurs, although some dermatologists suspect it is another autoimmune-based hair loss. The pathogenesis could be a destructive inflammatory process involving the upper outer root hair sheath including the area of the bulge which houses the follicular stem cells.


Figure 1–1 Pseudopelade of Brocq  (B Stevens)

Pseudo pelade
Figure 1–2 Pseudopelade of Brocq  (B Stevens)


3. History and Epidemiology
Neumann first described the condition in 1869, appreciating its similarity to alopecia areata; later in 1888 Brocq described it as pseudopelade (Pelade is the French term for alopecia areata). The term literally means „pseudo alopecia areata” due to similarities with that condition.
Nature of disease has been debated. Opinions are still divided as to whether PB is a specific disease entity or just the end-stage appearance of other scarring disorders, particularly LPP, DLE and CCLE.
Pseudopelade of Brocq is a scalp disorder of middle-aged adults of both sexes but most often seen in woman. The true prevalence of pseudopelade of Brocq in the general population is unknown, but it would appear to be very uncommon. Pseudopelade of Brocq is more common in whites. There are rare familian cases with onset of disease in childhood or adolescence.
4. Pathogenesis
The following two types of pseudopelade of Brocq are recognized nowadays:

  • Primary idiopathic pseudopelade. Pathophysiology unknown; usually a short, early phase of inflammation, evidenced on histophatology by massive, lymphocyte-mediated follicular destruction by apoptosis, has been proposed to account for a general lack of inflammation seen on exam in most cases.
  • Burnt-out or end-stage scarring alopecias (eg, LLP, DLE). Pathophysiology corresponds to underlying disease process.

5. Clinical features
Typically PB patient discovers discrete asymptomatic areas of scalp hair loss that most commonly affect the vertex and parietal scalp.
So PB manifests with multifocal, asymptomatic, small, smooth, flesh colored or white alopecic macules or patches without follicular hyperkeratosis or perifollicular inflammation.
Brocq described three patterns of pseudopelade:

  • scattered „petite plaques”,
  • large plaques and
  • a combination of these.

Over time, these plaques unites into larger patches with irregular borders that may be as large as several centimeters wide, surrounded by several satellite plaques. The patches are often atrophic with no inflammation clinically apparent; however, early lesions may show slight scale and follicular erythema. This kind of manifestation of skin plaques has been likened to “footprints in the snow” on the scalp. The follicle orifices are missing.
Typical of many forms of scarring alopecia, a few isolated hairs may remain within an otherwise smooth, shiny, denuded patch. Atrophy or degeneration of the hair follicles rather than scarring has been observed to be its primary feature.
The disease is slowly progressive with periods of activity and remission. New areas of alopecia develop over a period of months to years. However, PB often worsens in spurts, with periods of activity followed by periods of dormancy.
Progressive hair loss and the ability to extract anagen hairs easily with gentle traction are often the only evidence that the disease is active. Spontaneous resolution has been reported after 2 –18 years of disease activity.
Although some cases have been reported in children, the condition is primarily seen in adult women.
Pseudopelade of Brocq is a diagnosis of exclusion.

 

 6. Laboratory investigations and Pathology


Diagnosis of the condition is based on clinical, microbiological, histo-pathological and laboratory features.
Positive pull test may be only indication of active disease. It result for anagen hairs at the edge of the alopecia plaques.
Performance of he hair pull test is very simple. A dermatologist takes a few strands between their thumb and forefinger and pulls on them gently. Anagen or growing hairs should remain rooted in place while hairs in telogen phase should come out easily. By determining how many hairs were pulled and the number that came out, doctor can approximate activity of disease. This finding is rarely indicative in other forms of alopecia except in the case of active lichen planopilaris and occasionally in chronic cutaneous lupus erythematosus.

Laboratory Studies

Other than scalp biopsy, no laboratory test has been found useful in establishing the diagnosis of PB. If there are findings that suggest evidence of LED, antinuclear antibody testing would be appropriate.


Scalp biopsy

Two 4-mm deep punch biopsy specimens should be taken along the direction of the hair follicle. Specimens should ideally be obtained from a clinically well-established but active area of alopecia to include both normal and affected hair-bearing areas.
Pinkus described PB as a histologic and not a clinical entity. Routine histological examinations of classic PB display very non-specific findings.
Primary idiopathic pseudopelade is characterized by a contracted dermis with dense collagen and loss of space between collagen bundles. Elastic fibers are recoiled and appear thick. Broad fibrous tract remnants are noted with preservation of the elastic sheath. A variably dense perifollicular lymphocytic infiltrate appears in early stages of the disease. This is followed by atrophy of the follicular infundibular epithelium, concentric lamellar fibroplasia, sebaceous gland loss, and eventually complete destruction of the sebaceous unit.
In secondary pseudopelade, the histologic findings are those of a burnt-out scarring alopecia with absent hair follicles and fibrosis. Elastic tissue is absent in scarred areas.

7. Differential diagnosis


PB can mimic:

  • Morphea
  • Alopecia areata
  • Aplasia cutis congenita
  • Secondary syphilis
  • Central centrifugal cicatricial alopecia
  • Temporal triangular alopecia
  • Follicular degeneration syndrome
  • Tinea capitis
  • Lichen planopilaris
  • Discoid lupus erythematosus

It can be difficult to distinguish PB from alopecia areata, LPP, and DLE as clinical features are overlapping. This condition also shares features with CCCA. PB usually presents as unevenly bordered, typically atrophic plaques with irregularly shaped, widely distributed clusters of hair patches while in case of CCCA a symmetric patch is seen. CCCA shows a slow but steady disease progression, whereas the progression in the case of PB takes place in spurts.
The lesions of PB are often geometrically shaped, as opposed to the patches in alopecia areata.
Analysis of tissue biopsies of end stage lesions show that all follicular structures including sebaceous epithelium are destroyed to be replaced by collapsed and aggregated follicular adventitia. These changes are not diagnostic of PB and can be found in end-stage LPP as well. The Verhoeff-van Gieson (VVG) elastin stain can help in differentiating PB from other forms of alopecia which present common pathologic characteristics, but display distinct patterns of tissue staining.


8. PB Treatment
Primary Cicatricial Alopecias often result in permanent alopecia. The aim of treatment is to reduce symptoms and slow or stop progression of the scarring process. Early treatment is the key to preventing permanent hair loss.
When the lesions of PB are burnt out, treatment is not necessary. Unfortunately, PB can reactivate episodically and unpredictably. Monitoring therapeutic efficiency in such a condition, which is largely asymptomatic and has no obvious signs of inflammation, presents problems in defining a specific course of treatment and ascertaining therapeutic efficacy. If active inflammation is present, treatment may be reasonable and should focus on preventing disease progression. Even with treatment, PB may worsen. There is no widely accepted standard therapy defined as an effective treatment.
Varying degrees of success have been claimed with use of topical corticosteroids, intralesional triamcinolone acetonide, prednisone, hydroxy-chloroquine and isotretinoin in the treatment of PB.


Treatment

Level of evidence

Potent topical corticosteroids

Level of evidence = D

Hydroxychloroquine

Level of evidence = C

Antibiotics if Borrelia positive

Level of evidence = E

Thalidomide

?ineffective–Level of evidence = C

Table 8.1. Treatment.
Bulengo-Ransby and Headington reported a child who improved with hydroxychloroquine. They claimed, in their experience, that hydroxychloroquine is the only effective treatment for PB; but no further information was supplied. Tan et al. reported using hydroxychloroquine in 18 patients with PB without presenting details of response. New PB patches developed during hydroxychloroquine treatment in one report and no response was seen in all four patients enrolled in a 6-month prospective study (400mg daily).
Alzolibani et al from the University of British Columbia published the treatment recommendations based on their clinical experience:

  • Patients with active lesions with less than 10% scalp involvement:
    • Use a combination of a topical steroid (class I or II) applied twice daily with monthly intralesional corticosteroid injections with or without topical tacrolimus.
  • Patients who do not respond to topical treatment, those with greater than 10% scalp involvement, or those with rapidly progressive and aggressive disease:
    • Use hydroxychloroquine with or without oral prednisone initially. The oral prednisone is only used until the antimalarial has had time to take effect, and it should then be tapered appropriately over 2 months.

When starting any patient on hydroxychloroquine, baseline laboratory evaluations (CBC count, liver function testing) and an ophthalmologic (including retinal) examination should be preformed. Blood work should be repeated every 3 months. The ophthalmologic examination should be completed annually. Clinical improvement should be noted within 3-6 months. If the patient does not respond after 6 months of therapy with hydroxychloroquine, other treatment modalities should be pursued. If improvement is seen, continuing  it an additional year and then tapering the dose is reasonable. While Alzolibani et al refer to hydroxychloroquine as first-line systemic therapy, there concern exists that it is only useful in patients with underlying DLE.
Combination treatment of a potent topical steroid with an oral antihistamine (cetirizine) improved four patients with PB.
In one patient with PB and borreliosis, disease progression was halted with a 3-week course of cefotaxime. In a 6-month open trial, two patients were treated with thalidomide (100–200mg daily) without benefit.
Systemic therapy should be initiated and followed by a dermatologist who is familiar with the condition and experienced with using the above systemic medications.
The risks and benefits of systemic therapy should be closely considered by the clinician.

Surgical Care

Surgical correction has been used to treat scarring alopecia. As a general rule, the disease process should be dormant or stable for at least 1 year.
The progressive and intermittent nature of PB makes this determination difficult. In terms of unstable forms of alopecia, excision is the preferred surgical treatment. Factors such as scalp laxity and location are important when consulting a patient for alopecia reduction. The patient should clearly know that the surgical repair may be affected by future recurrences of their disease. Hair transplantation and flap procedures are less preferred surgical methods for treating unstable alopecia.
Some patients with extensive hair loss from PB can be advised to wear a wig or hair piece.

 

9. References.

 

 

 

 

 

 

 

 

 


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