eMedicine Specialties > Dermatology > Diseases of the Adnexa
Pseudopelade, Brocq
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 scalp2 ). 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.
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.12,13 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.
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
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References
Brocq L. Les folliculites et perifolliculites decalvantes. Bull Mem Soc Med Hop Paris. 1888;5:339-408.
Alzolibani AA, Kang H, Otberg N, Shapiro J. Pseudopelade of Brocq. Dermatol Ther. Jul-Aug 2008;21(4):257-63. [Medline].
Braun-Falco O, Plewig G, Wolff H, Burgdorf W, eds. Diseases of Hair. In: Dermatology. 2nd ed. New York, NY: Springer-Verlag; 2000:1120-21.
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.
Bolognia J, Jorizzo J, Rapini R. Alopecias. In: Dermatology. 2nd ed. Spain: Elsevier; 2008:1000.
Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol. Jun 2006;28(3):236-59. [Medline].
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].
Bulengo-Ransby SM, Headington JT. Pseudopelade of Brocq in a child. J Am Acad Dermatol. Nov 1990;23(5 Pt 1):944-5. [Medline].
Collier PM, James MP. Pseudopelade of Brocq occurring in two brothers in childhood. Clin Exp Dermatol. Jan 1994;19(1):61-4. [Medline].
Sperling LC, Solomon AR, Whiting DA. A new look at scarring alopecia. Arch Dermatol. Feb 2000;136(2):235-42. [Medline].
Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg. Mar 2006;25(1):41-50. [Medline].
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].
Khong JJ, Casson RJ, Huilgol SC, Selva D. Madarosis. Surv Ophthalmol. Nov-Dec 2006;51(6):550-60. [Medline].
Sahl WJ. Pseudopelade: an inherited alopecia. Int J Dermatol. Oct 1996;35(10):715-9. [Medline].
Pinkus H. Differential patterns of elastic fibers in scarring and non-scarring alopecias. J Cutan Pathol. Jun 1978;5(3):93-104. [Medline].
Bergner T, Braun-Falco O. Pseudopelade of Brocq. J Am Acad Dermatol. Nov 1991;25(5 Pt 1):865-6. [Medline].
Unger W, Unger R, Wesley C. The surgical treatment of cicatricial alopecia. Dermatol Ther. Jul-Aug 2008;21(4):295-311. [Medline].
Further Reading
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
