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Author: Leonard Sperling, MD, Chair, Professor, Department of Dermatology, Uniformed Services University of the Health Sciences

Leonard Sperling is a member of the following medical societies: American Academy of Dermatology

Editors: Evan R Farmer, MD, Professor of Dermatology, Johns Hopkins University School of Medicine, Clinical Professor of Pathology, Virginia Commonwealth University School of Medicine; Consulting Staff, Department of Dermatology, Johns Hopkins Hospital, VCU Health Services; Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA; Jeffrey J Miller, MD, Associate Professor, Department of Dermatology, Penn State University, Milton S Hershey Medical Center; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; William D James, MD, Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

Author and Editor Disclosure

Synonyms and related keywords: Brocq pseudopelade, hair loss, cicatricial alopecia, scarring alopecia

Background

Brocq used the term pseudopelade to describe a peculiar form of scarring alopecia resembling alopecia areata. (Pelade is the French term for alopecia areata.) This clinical entity is not a specific disease but a pattern of scarring alopecia, and the term pseudopelade of Brocq eventually should be abandoned. An additional source of confusion is the modern use of the term pseudopelade to describe a different form of scarring alopecia (ie, central, centrifugal, scarring alopecia [CCSA]).

Pseudopelade of Brocq is an end stage or clinical variant of various other forms of scarring alopecia and a diagnosis of exclusion. The same pattern of hair loss can be seen in burnt out (ie, no clinical or histologic evidence of inflammation) discoid lupus erythematosus (DLE), lichen planopilaris (LLP), and other forms of scarring alopecia. If a definitive diagnosis of DLE, LPP, or another condition can be made based on clinical, histologic, 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.

Frequency

International

Pseudopelade of Brocq is a very uncommon pattern of alopecia.

Race

Whites usually are affected more often.

Sex

Either sex can be affected.

Age

Adults usually are affected more often, although the condition has been reported in children.



History

The typical patient is surprised to discover discrete asymptomatic areas of scalp hair loss (Image 1). In many patients, the disease is slowly progressive; ie, new areas of alopecia develop over a period of months to years. However, the condition often worsens in spurts, with periods of activity followed by dormant periods. This is distinctly different from the slow but steady disease progression seen in several other forms of scarring alopecia. Disease progression in pseudopelade eventually ends spontaneously.

Physical

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

The classic description of "footprints in the snow" refers to dermal atrophy causing a slight depression below the surrounding normal scalp. In fact, many cases of pseudopelade do not demonstrate atrophy. Usually, only mild erythema and slight perifollicular scaling are present, and, often, no clinical evidence of inflammation is present. In fact, some authors would argue than any inflammation excludes traditional pseudopelade from the clinical differential diagnosis. Typical of many forms of scarring alopecia, a few isolated hairs may remain within an otherwise smooth, shiny, denuded patch. Include the nails and oral mucosa as well as the skin in physical examination to exclude evidence of other forms of scarring alopecia. Pseudopelade of Brocq is a diagnosis of exclusion.

Causes

Pseudopelade of Brocq is felt to be the end stage of several different forms of scarring alopecia, especially lichen planopilaris and chronic cutaneous lupus erythematosus. Therefore, the cause of pseudopelade of Brocq is linked to the etiology of the underlying skin disease.



Aplasia Cutis Congenita
Lupus Erythematosus, Discoid

Other Problems to be Considered

Central, centrifugal, scarring alopecia
Follicular degeneration syndrome
Lichen planopilaris
Temporal triangular alopecia



Lab Studies

  • No laboratory test has been found useful in establishing the diagnosis of pseudopelade of Brocq. If the history or physical examination suggests evidence of lupus erythematosus, antinuclear antibody testing would be appropriate.

Procedures

  • Scalp biopsy is required for determining both diagnosis and prognosis.
    • Ideally, the biopsy specimen is at least 4 mm in diameter and is sectioned transversely (horizontal to scalp surface).
    • At the very least, discovering that follicles have been replaced by connective tissue will confirm the diagnosis of permanent scarring alopecia.

Histologic Findings

The histopathologic findings of traditional pseudopelade have yet to be clearly defined. The criteria established by Pinkus, based on his experience and on the writings of several other authors, are not correlated in any way with clinical features. Thus, pseudopelade as described by Pinkus is a histologic and not a clinical entity. In most cases of traditional pseudopelade, the active lesion is elusive, and the typical histologic findings are those of a burnt-out scarring alopecia. The histologic findings of pseudopelade of the crown more recently described apply to CCCA and not pseudopelade of Brocq. A prospective study of pseudopelade of Brocq with sound clinical correlation has yet to be performed. In most cases, if an active lesion is sampled, the histologic findings are those of the primary process, eg, lichen planopilaris.



Medical Care

When lesions of pseudopelade of Brocq are burnt out, treatment is neither necessary nor possible. Unfortunately, the condition can reactivate episodically and unpredictably. If active inflammation is present, consider an alternative diagnosis, and potent topical corticosteroids, such as fluocinonide or clobetasol, can be tried.



The goals of pharmacotherapy are to reduce morbidity and prevent complications.

Drug Category: 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.

Drug NameClobetasol (Temovate)
DescriptionClass I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.
Adult DoseApply bid for up to 2 wk; not to exceed 50 g/wk
Pediatric DoseNot established
ContraindicationsDocumented hypersensitivity; viral or fungal skin infections
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsMay suppress adrenal function in prolonged therapy

Drug NameFluocinonide (Fluonex, Lidex)
DescriptionHigh-potency topical corticosteroid that inhibits cell proliferation; is immunosuppressive and anti-inflammatory.
Adult DoseApply sparingly bid/qid as severity warrants
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; herpes simplex infection; fungal, viral, or tubercular skin lesions
InteractionsNone reported
PregnancyC - Safety for use during pregnancy has not been established.
PrecautionsMay cause adverse systemic effects if used over large areas, denuded areas, on occlusive dressings, or during prolonged treatment periods



Prognosis

  • Unfortunately, the condition can reactivate episodically and unpredictably. There may be a single episode or numerous episodes extending over several decades.



Media file 1:  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.
Click to see larger pictureClick to see detailView Full Size Image
Media type:  Photo



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Pseudopelade, Brocq excerpt

Article Last Updated: Feb 22, 2007