Cicatricial alopecia
Encyclopedia
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. A variety of distributions are possible. 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. Cicatricial alopecia occurs in otherwise healthy men and women of all ages and is seen worldwide.
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, burn
s, radiation
, tumor
s, 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. The inflammation may predominantly involve lymphocyte
s or neutrophils. Cicatricial alopecias that predominantly involve lymph
ocytic 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
, tufted folliculitis
, and dissecting cellulitis. Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. A cicatricial alopecia with a mixed inflammatory infiltrate is folliculitis keloidalis.
s 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 men and women.
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. Central centrifugal cicatricial alopecia is the one exception; it 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.
s 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 follow your 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.
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.. 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, culture
s 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.
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.
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.
Treatment of the mixed group of cicatricial alopecias (folliculitis keloidalis) may include antimicrobial
s, isotretinoin, and anti-inflammatory medications.
You should discuss any treatment with your dermatologist, who will also explain 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.
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.
s can generally be used with any frequency desired, as long as the products are gentle and non-irritating to the scalp. Dermatologists may recommend specific shampoos and products to decrease scalp symptoms, scaling and inflammation. Hair pieces, wig
s, hats, and scarves may be used freely.
Hair follicle
A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....
, replace it with scar tissue
Scar tissue
Scar tissue can refer to:*Granulation tissue, a product of healing in major wounds*The tissue of a scar*"Scar Tissue", a Red Hot Chili Peppers song*Scar Tissue , the autobiography of Anthony Kiedis, lead singer of the Red Hot Chili Peppers...
, and cause permanent hair loss. A variety of distributions are possible. 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
Inflammation
Inflammation is part of the complex biological response of vascular tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. Inflammation is a protective attempt by the organism to remove the injurious stimuli and to initiate the healing process...
that destroys the follicle is below the skin surface and there is usually no "scar" seen on the scalp. Affected areas of the scalp
Scalp
The scalp is the anatomical area bordered by the face anteriorly and the neck to the sides and posteriorly.-Layers:It is usually described as having five layers, which can conveniently be remembered as a mnemonic:...
may show little signs of inflammation, or have redness, scaling
Scaling
Scaling may refer to:* Scaling , a linear transformation that enlarges or diminishes objects* Reduced scales of semiconductor device fabrication processes...
, increased or decreased pigmentation, pustules, or draining sinuses. Cicatricial alopecia occurs in otherwise healthy men and women of all ages and is seen worldwide.
Types of cicatricial alopecia
Cicatricial alopecias are classified as primary or secondary. This discussion is confined to the primary cicatricial alopecias in which the hair follicleHair follicle
A hair follicle is a skin organ that produces hair. Hair production occurs in phases, including a growth phase , and cessation phase , and a rest phase . Stem cells are principally responsible for the production of hair....
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, burn
Burn
A burn is an injury to flesh caused by heat, electricity, chemicals, light, radiation, or friction.Burn may also refer to:*Combustion*Burn , type of watercourses so named in Scotland and north-eastern England...
s, radiation
Radiation poisoning
Acute radiation syndrome also known as radiation poisoning, radiation sickness or radiation toxicity, is a constellation of health effects which occur within several months of exposure to high amounts of ionizing radiation...
, tumor
Tumor
A tumor or tumour is commonly used as a synonym for a neoplasm that appears enlarged in size. Tumor is not synonymous with cancer...
s, 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. The inflammation may predominantly involve lymphocyte
Lymphocyte
A lymphocyte is a type of white blood cell in the vertebrate immune system.Under the microscope, lymphocytes can be divided into large lymphocytes and small lymphocytes. Large granular lymphocytes include natural killer cells...
s or neutrophils. Cicatricial alopecias that predominantly involve lymph
Lymphatic system
The lymphoid system is the part of the immune system comprising a network of conduits called lymphatic vessels that carry a clear fluid called lymph unidirectionally toward the heart. Lymphoid tissue is found in many organs, particularly the lymph nodes, and in the lymphoid follicles associated...
ocytic inflammation include lichen planopilaris
Lichen planopilaris
Lichen planopilaris is a form of hair loss which involves scarring, a follicular form of lichen planus, and is considered to have an autoimmune cause...
, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq). Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans
Folliculitis decalvans
Folliculitis decalvans is an inflammatory alopecia that leads to bogginess or induration of involved parts of the scalp along with pustules, erosions, crusts, and scale. It begins at a central point and spreads outward, leaving scarring, sores, and hair loss in its wake...
, tufted folliculitis
Tufted folliculitis
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,...
, and dissecting cellulitis. Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. A cicatricial alopecia with a mixed inflammatory infiltrate is folliculitis keloidalis.
Causes
The cause of the various cicatricial alopecias is poorly understood. However, all cicatricial alopecias involve inflammation directed at the upper part of the hair follicle where the stem cellStem cell
This article is about the cell type. For the medical therapy, see Stem Cell TreatmentsStem cells are biological cells found in all multicellular organisms, that can divide and differentiate into diverse specialized cell types and can self-renew to produce more stem cells...
s and sebaceous gland
Sebaceous gland
The sebaceous glands are microscopic glands in the skin that secrete an oily/waxy matter, called sebum, to lubricate and waterproof the skin and hair of mammals...
(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
Contagious disease
A contagious disease is a subset category of infectious diseases , which are easily transmitted by physical contact with the person suffering the disease, or by their secretions or objects touched by them....
. In general, cicatricial alopecias are not associated with other illnesses, and usually occur in otherwise healthy men and women.
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
Family history (medicine)
In medicine, a family history consists of information about disorders from which the direct blood relatives of the patient have suffered. Genealogy typically includes very little of the medical history of the family, but the medical history could be considered a specific subset of the total history...
of a similar condition. Central centrifugal cicatricial alopecia is the one exception; it 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.
Symptoms
It is important to continue to watch for symptomSymptom
A symptom is a departure from normal function or feeling which is noticed by a patient, indicating the presence of disease or abnormality...
s 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 follow your cicatricial alopecia using these guidelines, and with the pull test. Photographs of the scalp
Scalp
The scalp is the anatomical area bordered by the face anteriorly and the neck to the sides and posteriorly.-Layers:It is usually described as having five layers, which can conveniently be remembered as a mnemonic:...
may be useful in monitoring the course of the disease and response to treatment.
Diagnosis
A scalp biopsyBiopsy
A biopsy is a medical test involving sampling of cells or tissues for examination. It is the medical removal of tissue from a living subject to determine the presence or extent of a disease. The tissue is generally examined under a microscope by a pathologist, and can also be analyzed chemically...
is essential for the diagnosis of cicatricial alopecia and is the necessary first step, as it can be hard to know the diagnosis
Diagnosis
Diagnosis is the identification of the nature and cause of anything. Diagnosis is used in many different disciplines with variations in the use of logics, analytics, and experience to determine the cause and effect relationships...
for sure without a biopsy.. 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, culture
Microbiological culture
A microbiological culture, or microbial culture, is a method of multiplying microbial organisms by letting them reproduce in predetermined culture media under controlled laboratory conditions. Microbial cultures are used to determine the type of organism, its abundance in the sample being tested,...
s 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.
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.
Treatment
As mentioned above, 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. 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
Lichen planopilaris
Lichen planopilaris is a form of hair loss which involves scarring, a follicular form of lichen planus, and is considered to have an autoimmune cause...
, 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
Hydroxychloroquine
Hydroxychloroquine is an antimalarial drug, sold under the trade names Plaquenil,Axemal, Dolquine, and Quensyl, also used to reduce inflammation in the treatment of rheumatoid arthritis and lupus...
, doxycycline
Doxycycline
Doxycycline INN is a member of the tetracycline antibiotics group, and is commonly used to treat a variety of infections. Doxycycline is a semisynthetic tetracycline invented and clinically developed in the early 1960s by Pfizer Inc. and marketed under the brand name Vibramycin. Vibramycin...
, mycophenolate mofetil
Mycophenolate mofetil
Mycophenolate mofetil is an immunosuppressant and prodrug of mycophenolic acid, used extensively in transplant medicine. It is a reversible inhibitor of inosine monophosphate dehydrogenase in purine biosynthesis, which is necessary for the growth of T cells and B cells...
, cyclosporine, or corticosteroids. Topical medications may include corticosteroids, tacrolimus
Tacrolimus
Tacrolimus is an immunosuppressive drug that is mainly used after allogeneic organ transplant to reduce the activity of the patient's immune system and so lower the risk of organ rejection...
, pimecrolimus
Pimecrolimus
Pimecrolimus is an immunomodulating agent used in the treatment of atopic dermatitis . It is currently available as a topical cream, once marketed by Novartis under the trade name Elidel.-Pharmacology:Pimecrolimus is an ascomycin macrolactam derivative...
, or Derma-Smoothe/FS scalp oil. Triamcinolone acetonide
Triamcinolone acetonide
Triamcinolone acetonide is a synthetic corticosteroid used to treat various skin conditions and to relieve the discomfort of mouth sores. It is a more potent type of triamcinolone, being about 8 times as effective as prednisone.-Medical use:...
, a corticosteroid, may be injected into inflamed, symptomatic areas of the scalp.
Treatment of the neutrophilic group of cicatricial alopecias (folliculitis decalvans
Folliculitis decalvans
Folliculitis decalvans is an inflammatory alopecia that leads to bogginess or induration of involved parts of the scalp along with pustules, erosions, crusts, and scale. It begins at a central point and spreads outward, leaving scarring, sores, and hair loss in its wake...
, tufted folliculitis
Tufted folliculitis
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,...
) 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
Isotretinoin
Isotretinoin, INN, is a medication used mostly for cystic acne. It was first developed for brain, pancreatic and other cancers. It is used to treat harlequin-type ichthyosis, a usually lethal skin disease, and lamellar ichthyosis. Its effects are systemic and nonselective...
in small doses may be helpful in treating dissecting cellulitis.
Treatment of the mixed group of cicatricial alopecias (folliculitis keloidalis) may include antimicrobial
Antimicrobial
An anti-microbial is a substance that kills or inhibits the growth of microorganisms such as bacteria, fungi, or protozoans. Antimicrobial drugs either kill microbes or prevent the growth of microbes...
s, isotretinoin, and anti-inflammatory medications.
You should discuss any treatment with your dermatologist, who will also explain potential side effects
Adverse effect
In medicine, an adverse effect is a harmful and undesired effect resulting from a medication or other intervention such as surgery.An adverse effect may be termed a "side effect", when judged to be secondary to a main or therapeutic effect. If it results from an unsuitable or incorrect dosage or...
, 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
Hair transplantation
Hair transplantation is a surgical technique that involves moving individual hair follicles from one part of the body to bald or balding parts . It is primarily used to treat male pattern baldness. It this case, grafts containing hair follicles that are genetically resistant to balding are...
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. MinoxidilMinoxidil
Minoxidil is an antihypertensive vasodilator medication which also slows or stops hair loss and promotes hair regrowth. Now off-patent, it is available over-the-counter for the treatment of androgenic alopecia. Minoxidil must be used indefinitely for continued support of existing hair follicles and...
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 shampooShampoo
Shampoo is a hair care product used for the removal of oils, dirt, skin particles, dandruff, environmental pollutants and other contaminant particles that gradually build up in hair...
s can generally be used with any frequency desired, as long as the products are gentle and non-irritating to the scalp. Dermatologists may recommend specific shampoos and products to decrease scalp symptoms, scaling and inflammation. Hair pieces, wig
Wig
A wig is a head of hair made from horsehair, human hair, wool, feathers, yak hair, buffalo hair, or synthetic materials which is worn on the head for fashion or various other aesthetic and stylistic reasons, including cultural and religious observance. The word wig is short for periwig and first...
s, hats, and scarves may be used freely.
External links
- Cicatricial Alopecia Research Foundation
- Clinical photos of folliculitis keloidalis from the Cicatricial Alopecia Research Foundation