eMedicine Specialties > Dermatology > Diseases of the Adnexa

Pseudopelade, Brocq

Kendall M Lane, MD, Resident Physician, Department of Dermatology, Naval Medical Center San Diego
Kimberly L Maino, MD, Head, Dermatologic Surgery/Oncology, Mohs Micrographic Surgery, Department of Dermatology, Naval Medical Center San Diego

Updated: Aug 31, 2009

Introduction

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 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 erythematous (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 2 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

• 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.

Frequency

International

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

Mortality/Morbidity

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

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.

Clinical

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 often worsens 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

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. 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 the occiput of a middle-aged white woman. This asymptomatic lesion was first discovered by the patient's hairdresser.

Causes

Most cases of pseudopelade of Brocq represent the end stage of LPP, DLE, or folliculitis decalvans. Idiopathic cases represent approximately 10% of patients and have a different histology. In support of pseudopelade as a primary disorder, rare familial cases have been reported.^{[9,14 ]}

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 deep 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 ]}

An active, but not recruiting, clinical trial is Expression of Fas Protein in Skin Biopsies of Participants With Scarring Alopecia.

Histologic Findings

The histopathologic criteria established by Pinkus were not correlated in any way with the clinical features.^{[15 ]}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.

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, some argue that it is only useful in patients with underlying discoid lupus erythematosus (DLE).^{[16 ]}

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 unstable forms of alopecia, excision is the preferred surgical treatment.^{[17 ]}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 unstable alopecia.

Consultations

• Dermatologist
• Plastic surgeon (if the patient is a candidate for surgical correction)

Medication

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

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)

Class I superpotent topical steroid; suppresses mitosis and increases synthesis of proteins that decrease inflammation and cause vasoconstriction.

Dosing

Adult

Apply bid for up to 2 wk; not to exceed 50 g/wk

Pediatric

Not established

Interactions

None reported

Contraindications

Documented hypersensitivity; viral or fungal skin infections

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May suppress adrenal function in prolonged therapy

Fluocinonide (Fluonex, Lidex)

High-potency topical corticosteroid that inhibits cell proliferation; is immunosuppressive and anti-inflammatory.

Dosing

Adult

Apply sparingly bid/qid as severity warrants

Pediatric

Administer as in adults

Interactions

None reported

Contraindications

Documented hypersensitivity; herpes simplex infection; fungal, viral, or tubercular skin lesions

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

May cause adverse systemic effects if used over large areas, denuded areas, on occlusive dressings, or during prolonged treatment periods

Antimalarial agents

Hydroxychloroquine (Plaquenil)

For treatment of DLE and SLE. Inhibits chemotaxis of eosinophils and locomotion of neutrophils; impairs complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate.

Dosing

Adult

Generalized dosing: 200-400 mg/d PO; not to exceed 6.5 mg/kg/d; 310 mg PO qd or bid for several wk depending on response; 155-310 mg/d for prolonged maintenance therapy
Dosing per Alzolibani et al recommendations: 200 mg PO bid; assess q3mo; if good response after 3-6 mo, continue for total of 9-12 mo then taper to 200 mg PO qd for 3-4 mo, then 200 mg PO qod for 3-4 mo; if no response after 6 mo, discontinuation is reasonable

Pediatric

Extremely rare in children; dosage not established

Interactions

Serum levels increase with cimetidine; magnesium trisilicate may decrease absorption

Contraindications

Absolute: Hypersensitivity, retinopathy from any cause
Relative: Pregnancy/lactation, retinal/visual-field change, severe blood dyscrasias, psoriasis, G-6-PD deficiency, significant hepatic dysfunction, myasthenia gravis, significant neurologic disease, long-term therapy in children

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic ophthalmologic examinations (including retinal, at least annually); test for muscle weakness; retinopathy, tinnitus, nerve deafness, skin eruption, headache, anorexia, nausea, vomiting, and diarrhea may occur.
CBC count and liver function testing should be performed prior to starting therapy and repeated q3mo

Follow-up

Prognosis

In reference to pseudopelade as a burnt-out from of alopecia discoid lupus erythematous (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 ]}

Patient Education

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

Multimedia

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.

References

1. Brocq L. Les folliculites et perifolliculites decalvantes. Bull Mem Soc Med Hop Paris. 1888;5:339-408.

2. Alzolibani AA, Kang H, Otberg N, Shapiro J. Pseudopelade of Brocq. Dermatol Ther. Jul-Aug 2008;21(4):257-63. [Medline].

3. Braun-Falco O, Plewig G, Wolff H, Burgdorf W, eds. Diseases of Hair. In: Dermatology. 2^nd ed. New York, NY: Springer-Verlag; 2000:1120-21.

4. Sperling LC. Brocq's alopecia (pseudopelade of Brocq) and "burnt out" scarring alopecia. In: Sperling LC, ed. An Atlas of Hair Pathology: With Clinical Correlations. London, England: Parthenon; 2003:115-8.

5. Bolognia J, Jorizzo J, Rapini R. Alopecias. In: Dermatology. 2^nd ed. Spain: Elsevier; 2008:1000.

6. Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol. Jun 2006;28(3):236-59. [Medline].

7. Otberg N, Wu WY, McElwee KJ, Shapiro J. Diagnosis and management of primary cicatricial alopecia: part I. Skinmed. Jan-Feb 2008;7(1):19-26. [Medline].

8. Bulengo-Ransby SM, Headington JT. Pseudopelade of Brocq in a child. J Am Acad Dermatol. Nov 1990;23(5 Pt 1):944-5. [Medline].

9. Collier PM, James MP. Pseudopelade of Brocq occurring in two brothers in childhood. Clin Exp Dermatol. Jan 1994;19(1):61-4. [Medline].

10. Sperling LC, Solomon AR, Whiting DA. A new look at scarring alopecia. Arch Dermatol. Feb 2000;136(2):235-42. [Medline].

11. Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg. Mar 2006;25(1):41-50. [Medline].

12. Madani S, Trotter MJ, Shapiro J. Pseudopelade of Brocq in beard area. J Am Acad Dermatol. May 2000;42(5 Pt 2):895-6. [Medline].

13. Khong JJ, Casson RJ, Huilgol SC, Selva D. Madarosis. Surv Ophthalmol. Nov-Dec 2006;51(6):550-60. [Medline].

14. Sahl WJ. Pseudopelade: an inherited alopecia. Int J Dermatol. Oct 1996;35(10):715-9. [Medline].

15. Pinkus H. Differential patterns of elastic fibers in scarring and non-scarring alopecias. J Cutan Pathol. Jun 1978;5(3):93-104. [Medline].

16. Bergner T, Braun-Falco O. Pseudopelade of Brocq. J Am Acad Dermatol. Nov 1991;25(5 Pt 1):865-6. [Medline].

17. Unger W, Unger R, Wesley C. The surgical treatment of cicatricial alopecia. Dermatol Ther. Jul-Aug 2008;21(4):295-311. [Medline].

Keywords

pseudopelade of Brocq, pseudopelade of Brocq, Brocq pseudopelade, hair loss, cicatricial alopecia, scarring alopecia, alopecia areata atrophicans, idiopathic scarring alopecia, fibrosing alopecia, alopecia cicatrisata, Brocq's alopecia

Contributor Information and Disclosures

Author

Kendall M Lane, MD, Resident Physician, Department of Dermatology, Naval Medical Center San Diego
Kendall M Lane, MD is a member of the following medical societies: American Academy of Dermatology, California Society of Dermatology and Dermatologic Surgery, and Pacific Dermatologic Association
Disclosure: Nothing to disclose.

Coauthor(s)

Kimberly L Maino, MD, Head, Dermatologic Surgery/Oncology, Mohs Micrographic Surgery, Department of Dermatology, Naval Medical Center San Diego
Kimberly L Maino, MD is a member of the following medical societies: American Academy of Dermatology, American College of Mohs Surgery, American Medical Association, and Women's Dermatologic Society
Disclosure: Nothing to disclose.

Medical Editor

Evan R Farmer, MD, Clinical Professor of Pathology and Dermatology, Department of Pathology, Virginia Commonwealth University School of Medicine
Evan R Farmer, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Medical Association, American Society of Dermatopathology, and International Society of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Richard P Vinson, MD, Assistant Clinical Professor, Department of Dermatology, Texas Tech University School of Medicine; Consulting Staff, Mountain View Dermatology, PA
Richard P Vinson, MD is a member of the following medical societies: American Academy of Dermatology, Association of Military Dermatologists, Texas Dermatological Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey J Miller, MD, Associate Professor of Dermatology, Penn State University College of Medicine; Staff Dermatologist, Penn State Milton S Hershey Medical Center
Jeffrey J Miller, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Association of Professors of Dermatology, North American Hair Research Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author, Leonard Sperling, MD, and previous Chief Editor, William D. James, MD, to the development and writing of this article.

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