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The term “cicatricial alopecia” refers to a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. The clinical course is highly variable and unpredictable. Hair loss may be slowly progressive over many years, without symptoms, and unnoticed for long periods. Or the hair loss may be rapidly destructive within months and associated with severe itching, pain and burning. The inflammation that destroys the follicle is below the skin surface and there is usually no “scar” seen on the scalp. Affected areas of the scalp may show little signs of inflammation, or have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Cicatricial alopecia occurs in otherwise healthy men and women of all ages, is not contagious, not hereditary and is seen worldwide.
The cause of the various cicatricial alopecia is poorly understood. However, all cicatricial alopecia involve inflammation directed at the hair follicle, usually the upper part of the follicle where the stem cells and sebaceous glands (oil glands) are located. If the stem cells and the sebaceous glands are destroyed, there is no possibility for regeneration of the hair follicle and resulting in permanent hair loss.
Research suggests that there is a loss of function of a “master regulator” called the peroxisome proliferator-activated receptor gamma, or PPAR gamma. PPAR gamma plays an important role in the preservation of hair follicle cells, including stem cells, and sebaceous glands. Decreased PPAR gamma leads to sebaceous gland dysfunction, which causes abnormal processing and buildup of “toxic” lipids. This abnormal buildup of lipids triggers inflammation that ultimately destroys the hair follicle.
Cicatricial alopecia affect both men and women of all ages and is rare in children, although some types may manifest in the teenage years. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecia. In general, they are not common. There have been a few reports of cicatricial alopecia occurring throughout a family and most patients with cicatricial alopecia have no family history of a similar condition.
Central centrifugal cicatricial alopecia (CCCA) affects women of African ancestry most commonly and may occur in several family members. Frontal fibrosing alopecia is seen most commonly in post-menopausal women but also occurs in young women and men. While it is possible to have more than one type of hair loss condition, non-scarring types of hair loss do not turn into scarring forms of hair loss.
In general, cicatricial alopecia is not associated with other illnesses and usually occur in healthy men and women. Patients with chronic cutaneous lupus erythematosus may have an increased personal and family history of autoimmune disorders
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