NORD gratefully acknowledges Vera H. Price, MD, Department of Dermatology, University of California, San Francisco, for her assistance in creating this report.
Cicatricial alopecias are classified as primary or secondary. This discussion is confined to the primary cicatricial alopecias, in which the hair follicle is the target of a destructive inflammatory process. 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. The inflammation that destroys the follicle is below the skin surface and there is usually no “scar” seen on the scalp, although the affected scalp is usually left bare and smooth without hair and without the usual pore markings.
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. 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.
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.
A scalp biopsy for the diagnosis of cicatricial alopecia is the necessary first step. 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. One or two biopsy specimens are taken, and ideally are examined after sectioning the skin samples both horizontally and vertically.
Clinical evaluation of the scalp is also important. Symptoms of itching, burning, pain, or tenderness usually signal ongoing activity. Signs of scalp inflammation include redness, scaling, and pustules. However, in some active cases there are few symptoms or signs and only the scalp biopsy demonstrates the active inflammation. The overall extent and pattern of hair loss is noted and, together with the biopsy findings, these enable the dermatologist to diagnose the specific cicatricial alopecia present. A hair pull test is performed to identify areas of active disease in which follicles are easily pulled out. The pulled hairs are mounted on a slide and the hair bulbs are viewed with a microscope to determine how many are growing (anagen) hairs and how many are resting (telogen) hairs. In addition, if pustules are present, cultures may be performed to identify which microbes, if any, may be contributing to the inflammation. A thorough evaluation that includes all of these parameters is important in diagnosing a cicatricial alopecia and in identifying features in individual patients that will help the selection of therapy.
Evaluation should be done by a dermatologist with a special interest or expertise in scalp and hair disorders, and who is familiar with current diagnostic methods and therapies.
Primary cicatricial alopecias are classified by the predominant type of inflammatory cells that attack the hair follicles (i.e., lymphocytes, neutrophils, or mixed inflammatory cells), and treatment strategies are different for each subtype and each patient.
Treatment of the lymphocytic group of cicatricial alopecias including lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq) involves use of anti-inflammatory medications. The goal of treatment is to decrease or eliminate the lymphocytic inflammatory cells that are attacking and destroying the hair follicle. Oral medications may include hydroxychloroquine, doxycycline, mycophenolate mofetil, cyclosporine, or pioglitazone. Topical medications may include corticosteroids, topical tacrolimus, topical pimecrolimus, or Derma-Smoothe/FS scalp oil. Triamcinolone acetonide (a corticosteroid) may be injected into inflamed, symptomatic areas of the scalp.
Treatment of the neutrophilic group of cicatricial alopecias (folliculitis decalvans, tufted folliculitis) is directed at eliminating the predominant microbes that are invariably involved in the inflammatory process. Oral antibiotics are the mainstay of therapy. Topical antibiotics and anti-inflammatory medications may be used to supplement the oral antibiotics.
Treatment of the mixed group of cicatricial alopecias (dissecting cellulitis, folliculitis keloidalis) may include antimicrobials, anti-inflammatory medications, isotretinoin (starting dose must be small). Infliximab may be helpful in treatment-resistant dissecting cellulitis.
The course of cicatricial alopecia is usually prolonged. Treatment is continued until the symptoms and signs of scalp inflammation are decreased, and progression of the condition has been controlled. Itching, burning, pain, and tenderness, and scalp redness, scaling, and/or pustules, can usually be controlled by current treatments. Unfortunately, the progression of the hair loss may continue silently even when the symptoms and signs are cleared. Cicatricial alopecia may reactivate after a quiet period and treatment may have to be repeated.
Surgical treatment for cosmetic benefit is an option in some cases after the disease has been inactive for one to two or more years. Hair restoration surgery or scalp reduction may be considered in these instances.
Hair will not regrow once the follicle is destroyed. However, it may be possible to treat the inflammation in and around surrounding follicles before they are destroyed, and for this reason it is important to begin treatment early to control the inflammatory process. Minoxidil solution or foam (2% or 5%) applied twice daily to the scalp may be helpful to stimulate any small, remaining, unscarred follicles. The progression of hair loss is unpredictable. In some cases, progression is slow and minimal, and in other cases progression can be rapid and extensive. Usually there is sufficient hair remaining to cover the affected scalp areas; relatively few patients require a hair piece.
Hair care products and shampoos are generally safe as long as they are non-irritating to the scalp. A dermatologist can recommend specific shampoos and products to decrease scalp symptoms, scaling and inflammation and will recommend frequency of their use. Hair pieces, wigs, hats, and scarves are all safe, will not aggravate your condition, and may be used freely.
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