Scarring hair loss

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Scarring hair loss
Other namesscarring alopecia
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg

Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss.

Contents

It can be caused by a diverse group of rare disorders that destroy the hair follicle, replace it with scar tissue, and cause permanent hair loss. A variety of distributions are possible. [1] In some cases, hair loss is gradual, without symptoms, and is unnoticed for long periods. In other cases, hair loss is associated with severe itching, burning and pain and is rapidly progressive. 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. Scarring hair loss occurs in otherwise healthy people of all ages and is seen worldwide.

Signs and symptoms

It is important to continue to watch for symptoms and signs of active disease during and after treatment to ensure that the disease is responding adequately and has not re-activated after therapy has been discontinued. Response to therapy may be indicated by the resolution of scalp symptoms such as itching, pain, tenderness, or burning, by improvement in the signs of scalp inflammation such as decreased redness, scaling or pustules, and by halting or slowing the progression of hair loss. A dermatologist can document and monitor a patient's cicatricial alopecia using these guidelines, and with the pull test. Photographs of the scalp may be useful in monitoring the course of the disease and response to treatment.

Causes

The cause of the various cicatricial alopecias is poorly understood. [2] However, all 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 then no possibility for regeneration of the hair follicle, and permanent hair loss results.

Cicatricial alopecias are not contagious. In general, cicatricial alopecias are not associated with other illnesses, and usually occur in otherwise healthy people.

Cicatricial alopecias affect both men and women, most commonly adults, although all ages may be affected. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecias. In general, they are not common.

The majority of patients with cicatricial alopecia have no family history of a similar condition. The one exception is Central centrifugal cicatricial alopecia, which primarily affects women of African ancestry and may occur in several women in the same family. 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.

Diagnosis

A scalp biopsy is essential for the diagnosis of cicatricial alopecia and is the necessary first step, as it can be hard to know the diagnosis for sure without a biopsy. [3] Findings of the scalp 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.

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 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 sometimes photographed for future comparison. A hair "pull test" is performed to see if growing, or anagen , where hairs are pulled out easily. The pulled hairs are mounted on a slide and the hair bulbs are viewed with a light microscope to determine how many are growing hairs and how many are resting hairs. In addition, if pustules are present, cultures are taken 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.

New diagnostic techniques, such as trichoscopy may be used for non-invasive differential diagnosis of cicatricial alopecia.

Diagnosis and treatment of cicatricial alopecias is often challenging. For this reason, it is helpful to be evaluated by a dermatologist with a special interest or expertise in scalp and hair disorders, and who is familiar with current diagnostic methods and therapies.

Classification

Boggy, suppurative nodule with patchy hair loss typical of dissecting cellulitis of the scalp. Dissecting cellulitis of the scalp - perifolliculitis capitis abscedens et suffodiens - Hoffman disease - 40yo male of African descent.jpg
Boggy, suppurative nodule with patchy hair loss typical of dissecting cellulitis of the scalp.

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 the 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, tumors, or traction.

Primary cicatricial alopecias are further classified by the type of inflammatory cells that destroy the hair follicle during the active stage of the disease. [4] The inflammation may predominantly involve lymphocytes or neutrophils. Cicatricial alopecias that predominantly involve lymphocytic inflammation include lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq). [5] Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans, tufted folliculitis, and dissecting cellulitis of the scalp. [6] Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. A cicatricial alopecia with a mixed inflammatory infiltrate is folliculitis keloidalis.

Treatment

As mentioned above, primary cicatricial alopecias are classified by the predominant type of inflammatory cells that attack the hair follicles, such as lymphocytes, neutrophils, or mixed inflammatory cells. Treatment strategies are different for each subtype and detailed treatment options are beyond the scope of this discussion. However, certain general principals are reviewed below.

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 corticosteroids. Topical medications may include corticosteroids, tacrolimus, 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 pathogenic microbes that are invariably involved in the inflammatory process. Oral antibiotics are the mainstay of therapy, and topical antibiotics may be used to supplement the oral antibiotics. In dissecting cellulitis, pathogenic microbes are not usually present. Isotretinoin in small doses may be helpful in treating dissecting cellulitis. [6]

Treatment of the mixed group of cicatricial alopecias (folliculitis keloidalis) may include antimicrobials, isotretinoin, and anti-inflammatory medications.

Patients are recommended to discuss any treatment with a dermatologist, who also explains potential side effects, as well as laboratory tests that are needed before starting treatment and sometimes are monitored during treatment.

The course of cicatricial alopecia is usually prolonged. Treatment is continued until the symptoms and signs of scalp inflammation are controlled, and progression of the condition has been slowed. In other words, itching, pain, tenderness, and burning have cleared, scalp redness, scaling, and/or pustules are no longer present, and the progression of the hair loss has been stopped or slowed. Treatment may then be stopped. Unfortunately, the cicatricial alopecias 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 regrowth

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 as early as possible to halt the inflammatory process. Minoxidil solution (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 there is always sufficient hair remaining to cover the affected scalp areas; in other cases, progression can be rapid and extensive.

Hair care

Hair care products and shampoos can generally be used with any frequency desired, as long as the products are gentle and non-irritating to the scalp. Hair pieces, wigs, hats, and scarves may be used freely.

See also

Related Research Articles

<span class="mw-page-title-main">Alopecia areata</span> Condition in which hair is lost from some or all areas of the body

Alopecia areata, also known as spot baldness, is a condition in which hair is lost from some or all areas of the body. Often, it results in a few bald spots on the scalp, each about the size of a coin. Psychological stress and illness are possible factors in bringing on alopecia areata in individuals at risk, but in most cases there is no obvious trigger. People are generally otherwise healthy. In a few cases, all the hair on the scalp is lost, or all body hair is lost. Hair loss can be permanent, or temporary. It is distinct from pattern hair loss, which is common among males.

<span class="mw-page-title-main">Hair loss</span> Loss of hair from the head or body

Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarring is not usually present. Hair loss in some people causes psychological distress.

<span class="mw-page-title-main">Folliculitis</span> Human and animal disease of follicles

Folliculitis is the infection and inflammation of one or more hair follicles. The condition may occur anywhere on hair-covered skin. The rash may appear as pimples that come to white tips on the face, chest, back, arms, legs, buttocks, or head.

<span class="mw-page-title-main">Demodicosis</span> Medical condition

Demodicosis, also called Demodex folliculitis in humans and demodectic mange or red mange in animals, is caused by a sensitivity to and overpopulation of Demodex spp. as the host's immune system is unable to keep the mites under control.

<span class="mw-page-title-main">Telogen effluvium</span> Medical condition

Telogen effluvium is a scalp disorder characterized by the thinning or shedding of hair resulting from the early entry of hair in the telogen phase. It is in this phase that telogen hairs begin to shed at an increased rate, where normally the approximate rate of hair loss is 125 hairs per day.

<span class="mw-page-title-main">Tinea capitis</span> Cutaneous fungal infection of the scalp

Tinea capitis is a cutaneous fungal infection (dermatophytosis) of the scalp. The disease is primarily caused by dermatophytes in the genera Trichophyton and Microsporum that invade the hair shaft. The clinical presentation is typically single or multiple patches of hair loss, sometimes with a 'black dot' pattern, that may be accompanied by inflammation, scaling, pustules, and itching. Uncommon in adults, tinea capitis is predominantly seen in pre-pubertal children, more often boys than girls.

Eosinophilic folliculitis is an itchy rash with an unknown cause that is most common among individuals with HIV, though it can occur in HIV-negative individuals where it is known by the eponym Ofuji disease. EF consists of itchy red bumps (papules) centered on hair follicles and typically found on the upper body, sparing the abdomen and legs. The name eosinophilic folliculitis refers to the predominant immune cells associated with the disease (eosinophils) and the involvement of the hair follicles.

<span class="mw-page-title-main">Feline acne</span> Medical condition

Feline acne is a problem seen in cats primarily involving the formation of blackheads accompanied by inflammation on the cat's chin and surrounding areas that can cause lesions, alopecia, and crusty sores. In many cases symptoms are mild and the disease does not require treatment. Mild cases will resemble dirt on the cat's chin, but the "dirt" will not brush off. More severe cases, however, may respond slowly to treatment and seriously detract from the health and appearance of the cat. Feline acne can affect cats of any age, sex or breed, although Persian cats are also likely to develop acne on the face and in the skin folds. This problem can happen once, be reoccurring, or even persistent throughout the cat's life.

<span class="mw-page-title-main">Discoid lupus erythematosus</span> Autoimmune skin condition

Discoid lupus erythematosus is the most common type of chronic cutaneous lupus (CCLE), an autoimmune skin condition on the lupus erythematosus spectrum of illnesses. It presents with red, painful, inflamed and coin-shaped patches of skin with a scaly and crusty appearance, most often on the scalp, cheeks, and ears. Hair loss may occur if the lesions are on the scalp. The lesions can then develop severe scarring, and the centre areas may appear lighter in color with a rim darker than the normal skin. These lesions can last for years without treatment.

<span class="mw-page-title-main">Dissecting cellulitis of the scalp</span> Medical condition

Dissecting cellulitis of the scalp, also known as dissecting folliculitis, perifolliculitis capitis abscedens et suffodiens of Hoffman, perifolliculitis abscedens et suffodiens, or folliculitis abscedens et suffodiens, is an inflammatory condition of the scalp that can lead to scarring alopecia, which begins with deep inflammatory nodules, primarily over occiput, that progresses to coalescing regions of boggy scalp. Boggy tissue has a high fluid level that results in a spongy feeling. Isotretinoin proves to be the medicine of choice for the treatment of the disease.

Anagen effluvium is the pathologic loss of anagen or growth-phase hairs. Classically, it is caused by radiation therapy to the head and systemic chemotherapy, especially with alkylating agents.

Central centrifugal cicatricial alopecia (CCCA), is a type of alopecia first noticed in African Americans in the 1950s and reported by LoPresti et al. in 1968 as a result of application of petrolatum followed by a stove-heated iron comb. The original theory was that the hot petrolatum would travel down to the hair root, burn the follicle, and after repetitive injury scarring would result. Later CCCA was realized to affect men and women without a history significant for use of such styling techniques. Consequently, the terms "follicular degeneration syndrome" per Sperling and Sau in 1992 and then CCCA per Olsent et al. in 2003 were evolved. Plausible contributing factors may include other African-American styling techniques such as relaxers, tight braids, heavy extensions, certain oils, gels or pomades.

<span class="mw-page-title-main">Folliculitis decalvans</span> Medical condition

Folliculitis decalvans is an inflammation of the hair follicle that leads to bogginess or induration of involved parts of the scalp along with pustules, erosions, crusts, ulcers, and scale. It begins at a central point and spreads outward, leaving scarring, sores, and, due to the inflammation, hair loss in its wake. No permanent cure has been found for this condition, but there is promise in a regimen of dual therapy with rifampin 300 mg twice daily and clindamycin 300 mg twice daily. This new treatment can be used to control the condition, and tests have indicated that after 3 to 5 months long uninterrupted courses of treatment, many patients have seen limited to no recurrence.

Pruritic folliculitis of pregnancy is a skin condition that occurs in one in 3000 people, about 0.2% of cases, who are in their second to third trimester of pregnancy where the hair follicle becomes inflamed or infected, resulting in a pus filled bump. Some dermatologic conditions aside from pruritic folliculitis during pregnancy include "pruritic urticarial papules and plaques of pregnancy, atopic eruption of pregnancy, pemphigoid gestationis, intrahepatic cholestasis of pregnancy, and pustular psoriasis of pregnancy". This pruritic folliculitis of pregnancy differs from typical pruritic folliculitis; in pregnancy, it is characterized by sterile hair follicles becoming inflamed mainly involving the trunk, contrasting how typical pruritic folliculitis is mainly localized on "the upper back, shoulders, and chest." This condition was first observed after some pregnant individuals showed signs of folliculitis that were different than seen before. The inflammation was thought to be caused by hormonal imbalance, infection from bacteria, fungi, viruses or even an ingrown hair. However, there is no known definitive cause as of yet. These bumps usually begin on the belly and then spread to upper regions of the body as well as the thighs. This condition does not harm the fetus or the mother and usually resolves after delivery of the baby. The rate of incidence could possibly be higher but due to the unknown etiology of the condition, misdiagnosis, and varying levels of severity, it is difficult to differentiate. Pruritic folliculitis of pregnancy is currently classified as atopic eruption of pregnancy (AEP) in a retrospective study done in 2006 that compared this condition to eczema and prurigo of pregnancy, which occurred in 49.7% and 0.8% of cases respectively, with eczema clearly being more frequent. Unlike typical pruritic folliculitis which does not resolve on its own, pruritic folliculitis of pregnancy clears spontaneously on delivery or in postpartum period. Fortunately, pruritic folliculitis of pregnancy has no mortality effects or significant adverse effects on the mother or on the fetus.

Tufted folliculitis presents with doll's hair-like bundling of follicular units, and is seen in a wide range of scarring conditions including chronic staphylococcal infection, chronic lupus erythematosus, lichen planopilaris, Graham-Little syndrome, folliculitis decalvans, acne keloidalis nuchae, immunobullous disorders, and dissecting cellulitis.

Non scarring hair loss, also known as noncicatricial alopecia is the loss of hair without any scarring being present. There is typically little inflammation and irritation, but hair loss is significant. This is in contrast to scarring hair loss during which hair follicles are replaced with scar tissue as a result of inflammation. Hair loss may be spread throughout the scalp (diffuse) or at certain spots (focal). The loss may be sudden or gradual with accompanying stress.

Alopecia mucinosa is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp, neck and face. This can also be present on the body as a follicular mucinosis and may represent a systemic disease.

<span class="mw-page-title-main">Frontal fibrosing alopecia</span> Medical condition

Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline. It is considered to be a clinical variant of lichen planopilaris.

<span class="mw-page-title-main">Fungal folliculitis</span> Inflammation of hair follicles due to fungal infection

Majocchi's granuloma is a skin condition characterized by deep, pustular plaques, and is a form of tinea corporis. It is a localized form of fungal folliculitis. Lesions often have a pink and scaly central component with pustules or folliculocentric papules at the periphery. The name comes from Professor Domenico Majocchi, who discovered the disorder in 1883. Majocchi was a professor of dermatology at the University of Parma and later the University of Bologna. The most common dermatophyte is called Trichophyton rubrum.

References

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  6. 1 2 Alexis, Andrew F. "Dissecting cellulitis of the scalp". uptodate.com. Retrieved 2021-01-06.{{cite web}}: CS1 maint: url-status (link)