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