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Cicatricial Alopecia: An Approach to Diagnosis and Management

Scarring alopecia is a group of inflammatory disorders in which there is permanent destruction of the pilosebaceous unit. In primary cicatricial alopecia, the hair follicle is the primary target of destruction by inflammation. In secondary cicatricial alopecia, the follicular destruction is incidental to a nonfollicular process such as infection, tumor, burn, radiation, or traction. Primary cicatricial alopecias are rare. The annual incidence rate of lichen planopilaris LPP in 4 hair loss centers in the United States varied from 1.

Central centrifugal cicatricial alopecia - an approach to diagnosis and management.

Scarring alopecia occurs when there is inflammation and destruction of the hair follicles leading to fibrous tissue formation. Hair loss in scarring alopecia is irreversible because the inflammatory infiltrate results in destruction of the hair follicle stem cells and the sebaceous glands. The inflammatory infiltrates are either predominantly lymphocytic, neutrophilic, or mixed. These differences are used to classify the scarring alopecias. Central centrifugal cicatricial alopecia CCCA. Scarring alopecias can vary by distribution and appearance.

Most patients will need a biopsy to confirm the clinical impression and determine the specific type of alopecia. This div only appears when the trigger link is hovered over. In secondary cicatricial alopecias, destruction of the hair follicle is incidental to a non-follicle-directed process or external injury, such as severe infections, burns, radiation, or tumors. Primary 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. Hair loss can be gradual, without symptoms, and unnoticed for long periods.

In other cases, the hair loss may be associated with severe itching, pain and burning, and progress rapidly. Cicatricial alopecia occurs in otherwise healthy men and women of all ages and is seen worldwide. Affected areas of the scalp may have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Other cases may show little signs of inflammation. Cicatricial alopecias are further classified by the type of inflammatory cells that destroy the hair follicle during the active stage of the disease.

The inflammation may involve predominantly lymphocytes or neutrophils. Cicatricial alopecias that involve predominantly lymphocytic inflammation include lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade Brocq. Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans and tufted folliculitis.

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Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. Cicatricial alopecias with a mixed inflammatory infiltrate include dissecting cellulitis and folliculitis keloidalis.


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The cause of the various cicatricial alopecias is not well understood. However, all types of cicatricial alopecias involve inflammation directed at the upper part of the hair follicle where the stem cells and sebaceous gland oil gland are located. If the stem cells and sebaceous gland are destroyed, there is no possibility for regeneration of the hair follicle, leading to permanent hair loss.

Cicatricial alopecias are not contagious. Cicatricial alopecias affect healthy men and women of all ages, although primary cicatricial alopecia is not usually seen in children. Cicatricial alopecias occur worldwide. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecias. In general, they are not common. There have been a few reports of cicatricial alopecia occurring in a family. However, the majority of patients with cicatricial alopecia have no family history of a similar condition.

Central centrifugal alopecia most commonly affects women of African ancestry and may occur in more than one family member. Dissecting cellulitis looks like deep cystic acne involving the scalp, and it occurs primarily in dark-skinned men.

While it is possible to have more than one type of hair loss condition, non-scarring forms of hair loss do not turn into scarring forms of hair loss. Symptoms of the following disorder can be similar to those of cicatricial alopecia. Comparisons may be useful for a differential diagnosis:. Chronic cutaneous lupus erythematosus CCLE is predominantly a cutaneous disease with few systemic complications. Scarring and disfiguring changes in the skin discoid lesons are commonly seen in this disease. It occurs more frequently in females than males and more commonly in adults than children.

A scalp biopsy for the diagnosis of cicatricial alopecia is the necessary first step.

Trichoscopy of Primary Cicatricial Alopecia, EDOJ11(1):5

Findings of the biopsy, including the type of inflammation present, location and amount of inflammation, and other changes in the scalp, are necessary to diagnose the type of cicatricial alopecia, to determine the degree of activity, and to select appropriate therapy. The biopsy specimen is taken with a biopsy punch, which is an instrument that removes a sample of skin about the size and shape of a small pencil eraser, after anesthetizing the local area.

Cicatricial alopecia is a trichologic emergency state which requires a fast and confident confirmation of diagnosis, as well as aggressive treatment in the active stage of the disease to guard against permanent destruction of hair follicles therefore trichoscopy may be applied as a quick and non-invasive method that helps in the differential diagnosis of diverse diseases leading to cicatricial alopecia.

To evaluate the potential benefit of trichoscopy in the clinical diagnosis of primary cicatricial alopecia.

Alopecia Hair Loss Treatment Results

The most characteristic dermoscopic findings in each disease were as follows: Trichoscopy is a noninvasive tool that significantly improves the accuracy of the diagnosis of PCA. Cicatricial alopecias are a group of intractable and uncommon hair loss disorders characterized by permanent hair follicle destruction [ 1 - 5 ]. The most typical clinical manifestation of cicatricial alopecia is the loss of visible follicular ostia in a scarring area [ 4 , 5 ]. The histopathological hallmark of a fully developed lesion is the replacement of the hair follicle structure by fibrous tissue [ 1 , 5 , 6 ].

Primary cicatricial alopecia PCA is a group of disorders, in which the hair follicle is the main target of destructive inflammation resulting in irreversible hair loss [ 4 , 5 , 7 - 9 ].

PCA were divided into subgroups depending on the predominating inflammatory infiltrates. The loss of follicular ostia, which is the most characteristic feature of PCA, may not be clinically evident in some cases, but could be clearly visualized under trichoscopy. Indeed, trichoscopy significantly improves the accuracy of the diagnosis of PCA [ 4 ].

Other PCA-associated signs, such as perifollicular erythema or scale, hair tufting are also detectable [ 10 ].


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Thus trichoscopy can helps clinicians assessing PCA disease activity [ 11 ]. Dermoscopic examination of the LPP patients revealed perifollicular scales in The dermoscopic finding of KFSD was black dots, follicular keratosis, short cut-off hairs, hypotrichosis, perifollicular scales and honeycomb appearance in addition to short cutoff lashes and follicular scales Figure 6. Cicatricial alopecia, also called scarring alopecia, represents a "trichologic emergency" because hair follicles are permanently destroyed so a fast and confident confirmation of the diagnosis, as well as aggressive treatment in the case of active disease, is crucial in the management of scarring alopecia [ 12 ].