Brocq Pseudopelade 

Updated: Mar 10, 2017
Author: Kendall M Egan, MD, FAAD; Chief Editor: William D James, MD 

Overview

Background

In 1888, Brocq used the term pseudopelade to describe a unique form of cicatricial alopecia resembling alopecia areata (pelade is the French term for alopecia areata).[1] Over the last century, this condition has been a source of controversy.

While some believe pseudopelade is a unique entity, most now believe that it is an end stage or clinical variant of various forms of cicatricial alopecia.[2] The same pattern of alopecia can be found in end-stage discoid lupus erythematosus (DLE), lichen planopilaris (LLP), and other forms of cicatricial alopecia. The confusion is further amplified by a difference in definition between different countries. For example, in Germany, all types of inflammatory cicatricial alopecia are included in the grouping of pseudopelade. In contrast, American dermatologists have used the term as a diagnosis of exclusion.[3]

Pseudopelade of Brocq is not a specific disease, but a pattern of cicatricial alopecia.[4] 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, but this has yet to be established with certainty.

Pathophysiology

The following two types of pseudopelade of Brocq are recognized:

  • Burnt-out or end-stage scarring alopecias (eg, LLP, DLE)[5] - Pathophysiology corresponds to underlying disease process

  • Primary idiopathic pseudopelade - Pathophysiology unknown

Pseudopelade of Brocq is considered end-stage permanent alopecia. The general pathogenesis of scarring alopecias has focused on the following theories of thought[6, 7] :

  • Stem cell failure: The bulge region of the hair follicle houses follicular stem cells. These cells are essential for hair growth. Direct damage to the bulge region may cause permanent scarring hair loss.

  • Sebaceous gland destruction: The sebaceous gland connects to the hair follicle just superior to where the inner root sheath degenerates. This degeneration is required for the hair shaft to exit the skin normally. The sebaceous gland may have a crucial role in this process.

Epidemiology

Frequency

The true prevalence of pseudopelade of Brocq in the general population is unknown, but it would appear to be very uncommon.

Race

Pseudopelade of Brocq is more common in whites.

Sex

Females are affected by pseudopelade of Brocq more often than males. The typical patient is a middle-aged woman with type 2 skin.

Age

Although pseudopelade of Brocq has been reported rarely in children,[8, 9] the onset most commonly occurs between ages 30 and 50 years.

Prognosis

In reference to pseudopelade as a burnt-out form of alopecia discoid lupus erythematosus (DLE) or lichen planopilaris (LLP), the prognosis depends on the underlying disease process.

Primary pseudopelade or idiopathic scarring alopecia can reactivate episodically and unpredictably. Episodes may be single or may be numerous extending over several decades. Some patients continue to have focal hair loss, while others progress to widespread alopecia. Rare cases of rapidly progressing pseudopelade have been reported.[2]  

Pseudopelade of Brocq patients may have emotional distress due to the progressive nature of the disorder and the poor response to treatment.

Patient Education

Information can be obtained from the Cicatricial Alopecia Research Foundation (C.A.R.F.).

 

Presentation

History

The typical pseudopelade of Brocq patient is surprised to discover discrete asymptomatic areas of scalp hair loss (most commonly affecting the vertex and parietal scalp[2] ). In many patients, pseudopelade of Brocq is slowly progressive (ie, new areas of alopecia develop over a period of months to years). However, pseudopelade of Brocq may worsen in spurts, with periods of activity followed by periods of dormancy. This is distinctly different from the slow but steady disease progression seen in several other forms of scarring alopecia.[10, 11] As the condition progresses, pseudopelade of Brocq patients may become emotionally distressed with the lack of treatment options and uncertain etiology of their condition. Disease progression in pseudopelade eventually ends spontaneously.

Physical Examination

Lesions of pseudopelade are randomly distributed, irregularly shaped, and often cluster in patches on the scalp. Cases with exclusive crown or vertex involvement actually may represent examples of burnt-out central cicatricial alopecia.[13] The individual lesion is hypopigmented (porcelain white is the classic description) and slightly depressed (atrophic). Pseudopelade of Brocq lesions often are shaped irregularly, as opposed to the round or oval patches usually seen in alopecia areata and most cases of central cicatricial alopecia.

Irregularly shaped patch of scarring alopecia on t Irregularly shaped patch of scarring alopecia on the occiput of a middle-aged white woman. This asymptomatic lesion was first discovered by the patient's hairdresser.

Typical of many forms of scarring alopecia, a few isolated hairs may remain within an otherwise smooth, shiny, denuded patch. Rare cases of pseudopelade have been reported to affect the beard or eyebrows in addition to the scalp.[14, 15] Include the nails and oral mucosa, as well as the skin, in the physical examination to exclude evidence of other forms of scarring alopecia. Pseudopelade of Brocq is a diagnosis of exclusion.

Dermatoscopic examination

An absence of follicular ostia is noted with dermoscopy.[16]

Causes

Most cases of pseudopelade of Brocq represent the end stage of LPP, DLE, or folliculitis decalvans.  In support of pseudopelade as a primary disorder, rare familial cases have been reported.[9, 12]

 

DDx

Differential Diagnoses

 

Workup

Laboratory Studies

Other than scalp biopsy, no laboratory test has been found useful in establishing the diagnosis of pseudopelade of Brocq. If the history or physical examination findings suggest evidence of lupus erythematosus, antinuclear antibody testing would be appropriate.

Procedures

Scalp biopsy

Two 4-mm punch biopsy specimens should be obtained from an orientation along the direction of the hair follicle. Specimens should ideally be taken from a clinically well-established but active area of alopecia to include both normal and affected hair-bearing areas. One punch biopsy specimen should be submitted for horizontal sectioning and one for vertical sections if possible, both stained with hematoxylin and eosin and elastic tissue stains. The second punch biopsy specimen can be bisected vertically to accommodate both direct immunofluorescence (DIF) and hematoxylin and eosin staining.[7]

Histologic Findings

The histopathologic criteria established by Pinkus were not correlated in any way with the clinical features.[17] Thus, pseudopelade as described by Pinkus is a histologic and not a clinical entity. 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. 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.

 

Treatment

Medical Care

When the lesions of pseudopelade of Brocq are burnt out, treatment is neither necessary nor possible. Unfortunately, pseudopelade of Brocq can reactivate episodically and unpredictably. If active inflammation is present, treatment may be reasonable and should focus on preventing disease progression. Even with treatment, pseudopelade of Brocq may worsen. Standardized treatment does not exist. Alzolibani et al from the University of British Columbia published the following treatment recommendations based on their clinical experience[2] :

  • 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.

Before starting any patient on hydroxychloroquine, baseline laboratory evaluations (glucose-6-phosphate dehydrogenase [G6PD], CBC count, liver function tests, Cr/BUN) and an ophthalmologic (including retinal) examination should be performed. Blood work should be repeated every 3 months (CBC count, liver function tests, Cr/BUN). The ophthalmologic examination should be completed every 6-12 months or as recommended by an ophthalmologist. The maximum daily dose is based on ideal body weight (6.5 mg/kg/day). 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, some argue that it is only useful in patients with underlying discoid lupus erythematosus (DLE).[18]  

Treatment with isotretinoin and mycophenolate mofetil (CellCept) have also been used separately, with limited success.[2] Frequent blood work and pregnancy testing are required for both medications.

Systemic therapy should be initiated and followed by a dermatologist who is familiar with the condition and experienced with using the above systemic medications. Pseudopelade, like most scarring alopecias, is difficult to treat and, in general, responds poorly to treatment. This should be taken into account when the clinician is determining treatment options. The risks and benefits of systemic therapy should be closely scrutinized by the prescribing 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 pseudopelade (unstable alopecia) makes this determination difficult.  In terms of stable forms of cicatricial alopecia, excision is the preferred surgical treatment.[19] Factors such as scalp laxity and location are important when considering 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 cicatricial alopecia.

Consultations

Consultations may include a dermatologist and a plastic surgeon (if the patient is a candidate for surgical correction).

 

Medication

Medication Summary

The goal of pharmacotherapy in pseudopelade of Brocq is to slow disease progression. Remember that no standard therapies are available for pseudopelade of Brocq.

Corticosteroids

Class Summary

Corticosteroids have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Clobetasol (Temovate)

Clobetasol is a class I superpotent topical steroid; it suppresses mitosis and increases the synthesis of proteins that decrease inflammation and cause vasoconstriction.

Fluocinonide (Fluonex, Lidex)

Fluocinonide is a high-potency topical corticosteroid that inhibits cell proliferation; it is immunosuppressive and anti-inflammatory.

Antimalarial agents

Hydroxychloroquine (Plaquenil)

Hydroxychloroquine inhibits chemotaxis of eosinophils and locomotion of neutrophils; it impairs complement-dependent antigen-antibody reactions.