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Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with
scarring A scar (or scar tissue) is an area of fibrous tissue that replaces normal skin after an injury. Scars result from the biological process of wound repair in the skin, as well as in other organs, and tissues of the body. Thus, scarring is a nat ...
. This is in contrast to non scarring hair loss. It can be caused by a diverse group of rare disorders that destroy the
hair follicle The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction betwee ...
, replace it with
scar tissue "Scar Tissue" is the first single from American rock band Red Hot Chili Peppers' seventh studio album, ''Californication'' (1999). Released on May 25, 1999, the song spent a then-record 16 consecutive weeks atop the US ''Billboard'' Hot Modern R ...
, and cause permanent hair loss. A variety of distributions are possible.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). Page 647. McGraw-Hill. . In some cases,
hair loss 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 gradual, without symptoms, and is unnoticed for long periods. In other cases, hair loss is associated with severe
itching An itch (also known as pruritus) is a sensation that causes a strong desire or reflex to scratch. Itches have resisted many attempts to be classified as any one type of sensory experience. Itches have many similarities to pain, and while both ...
, burning and pain and is rapidly progressive. The
inflammation Inflammation (from ) is part of the biological response of body tissues to harmful stimuli, such as pathogens, damaged cells, or irritants. The five cardinal signs are heat, pain, redness, swelling, and loss of function (Latin ''calor'', '' ...
that destroys the follicle is below the skin surface and there is usually no "scar" seen on the scalp. Affected areas of the
scalp The scalp is the area of the head where head hair grows. It is made up of skin, layers of connective and fibrous tissues, and the membrane of the skull. Anatomically, the scalp is part of the epicranium, a collection of structures covering th ...
may show little signs of inflammation, or have redness, scaling, increased or decreased
pigmentation A pigment is a powder used to add or alter color or change visual appearance. Pigments are completely or nearly insoluble and chemically unreactive in water or another medium; in contrast, dyes are colored substances which are soluble or go in ...
, pustules, or draining
sinuses Paranasal sinuses are a group of four paired air-filled spaces that surround the nasal cavity. The maxillary sinuses are located under the eyes; the frontal sinuses are above the eyes; the ethmoidal sinuses are between the eyes and the sphenoi ...
. 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 Signs and symptoms are diagnostic indications of an illness, injury, or condition. Signs are objective and externally observable; symptoms are a person's reported subjective experiences. A sign for example may be a higher or lower temperature ...
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 A skin condition, also known as cutaneous condition, is any medical condition that affects the integumentary system—the organ system that encloses the body and includes skin, nails, and related muscle and glands. The major function of this sys ...
, 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 The scalp is the area of the head where head hair grows. It is made up of skin, layers of connective and fibrous tissues, and the membrane of the skull. Anatomically, the scalp is part of the epicranium, a collection of structures covering th ...
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. However, all cicatricial alopecias involve inflammation directed at the upper part of the hair follicle where the
stem cell In multicellular organisms, stem cells are undifferentiated or partially differentiated cells that can change into various types of cells and proliferate indefinitely to produce more of the same stem cell. They are the earliest type of cell ...
s and
sebaceous gland A sebaceous gland or oil gland is a microscopic exocrine gland in the skin that opens into a hair follicle to secrete an oily or waxy matter, called sebum, which lubricates the hair and skin of mammals. In humans, sebaceous glands occur in ...
(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 Genealogy () is the study of families, family history, and the tracing of their lineages. Genealogists use oral interviews, historical records, genetic analysis, and other records to obtain information about a family and to demonstrate kins ...
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 A biopsy is a medical test commonly performed by a surgeon, interventional radiologist, an interventional radiologist, or an interventional cardiology, interventional cardiologist. The process involves the extraction of sampling (medicine), sample ...
is essential for the diagnosis of cicatricial alopecia and is the necessary first step, as it can be hard to know the
diagnosis Diagnosis (: diagnoses) is the identification of the nature and cause of a certain phenomenon. Diagnosis is used in a lot of different academic discipline, disciplines, with variations in the use of logic, analytics, and experience, to determine " ...
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 The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction between ho ...
'', where hairs are pulled out easily. The pulled hairs are mounted on a slide and the hair bulbs are viewed with a
light microscope The optical microscope, also referred to as a light microscope, is a type of microscope that commonly uses visible spectrum, visible light and a system of lens (optics), lenses to generate magnified images of small objects. Optical microscopes ...
to determine how many are growing hairs and how many are resting hairs. In addition, if pustules are present,
culture Culture ( ) is a concept that encompasses the social behavior, institutions, and Social norm, norms found in human societies, as well as the knowledge, beliefs, arts, laws, Social norm, customs, capabilities, Attitude (psychology), attitudes ...
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. New diagnostic techniques, such as
trichoscopy Trichoscopy is a method of hair and scalp evaluation and is used for diagnosing hair and scalp diseases. The method is based on dermoscopy. In trichoscopy hair and scalp structures may be visualized at many-fold magnification. Currently magnificat ...
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 Dermatology is the branch of medicine dealing with the skin.''Random House Webster's Unabridged Dictionary.'' Random House, Inc. 2001. Page 537. . It is a speciality with both medical and surgical aspects. A dermatologist is a specialist medi ...
with a special interest or expertise in scalp and hair disorders, and who is familiar with current diagnostic methods and therapies.


Classification

Cicatricial alopecias are classified as primary or secondary. This discussion is confined to the primary cicatricial alopecias in which the
hair follicle The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction betwee ...
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 An infection is the invasion of tissue (biology), tissues by pathogens, their multiplication, and the reaction of host (biology), host tissues to the infectious agent and the toxins they produce. An infectious disease, also known as a transmis ...
,
burn A burn is an injury to skin, or other tissues, caused by heat, electricity, chemicals, friction, or ionizing radiation (such as sunburn, caused by ultraviolet radiation). Most burns are due to heat from hot fluids (called scalding), soli ...
s,
radiation In physics, radiation is the emission or transmission of energy in the form of waves or particles through space or a material medium. This includes: * ''electromagnetic radiation'' consisting of photons, such as radio waves, microwaves, infr ...
,
tumor A neoplasm () is a type of abnormal and excessive growth of tissue. The process that occurs to form or produce a neoplasm is called neoplasia. The growth of a neoplasm is uncoordinated with that of the normal surrounding tissue, and persists ...
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 A lymphocyte is a type of white blood cell (leukocyte) in the immune system of most vertebrates. Lymphocytes include T cells (for cell-mediated and cytotoxic adaptive immunity), B cells (for humoral, antibody-driven adaptive immunity), an ...
s or
neutrophil Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in differe ...
s. Cicatricial alopecias that predominantly involve
lymph Lymph () is the fluid that flows through the lymphatic system, a system composed of lymph vessels (channels) and intervening lymph nodes whose function, like the venous system, is to return fluid from the tissues to be recirculated. At the ori ...
ocytic inflammation include
lichen planopilaris Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. It is characterized by polygonal, flat-topped, violaceous p ...
, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq). Cicatricial alopecias that are due to predominantly
neutrophilic Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in different ...
inflammation include folliculitis decalvans,
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 synd ...
, and
dissecting cellulitis of the scalp 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 ...
. Sometimes the inflammation shifts from a predominantly
neutrophilic Neutrophils are a type of phagocytic white blood cell and part of innate immunity. More specifically, they form the most abundant type of granulocytes and make up 40% to 70% of all white blood cells in humans. Their functions vary in different ...
process to a lymphocytic process. A cicatricial alopecia with a mixed inflammatory infiltrate is
folliculitis keloidalis 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. A ...
.


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 Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. It is characterized by polygonal, flat-topped, violaceous p ...
, 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, sold under the brand name Plaquenil among others, is a medication used to prevent and treat malaria in areas where malaria remains sensitive to chloroquine. Other uses include treatment of rheumatoid arthritis, lupus, an ...
,
doxycycline Doxycycline is a Broad-spectrum antibiotic, broad-spectrum antibiotic of the Tetracycline antibiotics, tetracycline class used in the treatment of infections caused by bacteria and certain parasites. It is used to treat pneumonia, bacterial p ...
,
mycophenolate mofetil Mycophenolic acid is an immunosuppressant medication used to prevent rejection following organ transplantation and to treat autoimmune conditions such as Crohn's disease and lupus. Specifically it is used following kidney, heart, and live ...
,
cyclosporine Ciclosporin, also spelled cyclosporine and cyclosporin, is a calcineurin inhibitor, used as an immunosuppressant medication. It is taken orally or intravenously for rheumatoid arthritis, psoriasis, Crohn's disease, nephrotic syndrome, ecz ...
, or
corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...
. Topical medications may include
corticosteroids Corticosteroids are a class of steroid hormones that are produced in the adrenal cortex of vertebrates, as well as the synthetic analogues of these hormones. Two main classes of corticosteroids, glucocorticoids and mineralocorticoids, are invol ...
,
tacrolimus Tacrolimus, sold under the brand name Prograf among others, is an immunosuppressive drug. After Allotransplantation, allogenic organ transplant, the risk of organ Transplant rejection, rejection is moderate. To lower the risk of organ rejectio ...
,
pimecrolimus Pimecrolimus is an immunosuppressant drug of the calcineurin inhibitor class used in the treatment of atopic dermatitis (eczema). It is available as a topical cream. It was developed and formerly marketed by Novartis under the trade name Elide ...
, or Derma-Smoothe/FS scalp oil.
Triamcinolone acetonide Triamcinolone acetonide, sold under the brand name Kenalog among others, is a synthetic compound, synthetic corticosteroid medication used topical medication, topically to treat various skin conditions, to relieve the discomfort of mouth sores, ...
, a corticosteroid, may be injected into inflamed, symptomatic areas of the scalp. Treatment of the neutrophilic group of cicatricial alopecias ( folliculitis decalvans,
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 synd ...
) 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, also known as 13-''cis''-retinoic acid and sold under the brand name Accutane among others, is a medication used to treat skin diseases like harlequin-type ichthyosis, and lamellar ichthyosis, and severe cystic acne or moderat ...
in small doses may be helpful in treating dissecting cellulitis. Treatment of the mixed group of cicatricial alopecias (folliculitis keloidalis) may include
antimicrobials An antimicrobial is an agent that kills microorganisms (microbicide) or stops their growth (bacteriostatic agent). Antimicrobial medicines can be grouped according to the microorganisms they are used to treat. For example, antibiotics are used aga ...
, isotretinoin, and
anti-inflammatory medication Anti-inflammatory is the property of a substance or treatment that reduces inflammation, fever or swelling. Anti-inflammatory drugs, also called anti-inflammatories, make up about half of analgesics. These drugs reduce pain by inhibiting mechani ...
s. Patients are recommended to discuss any treatment with a dermatologist, who also explains potential
side effects In medicine, a side effect is an effect of the use of a medicinal drug or other treatment, usually adverse but sometimes beneficial, that is unintended. Herbal and traditional medicines also have side effects. A drug or procedure usually used ...
, 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 Minoxidil is a medication used for the treatment of high blood pressure and pattern hair loss. It is an antihypertensive and a vasodilator. It is available as a generic medication by prescription in oral administration, oral tablet (pharmacy), ...
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 Shampoo () is a hair care product, typically in the form of a Viscosity, viscous liquid, that is formulated to be used for cleaning (scalp) hair. Less commonly, it is available in solid bar format. ("Dry shampoo" is a separate product.) Shamp ...
can generally be used with any frequency desired, as long as the products are gentle and non-irritating to the scalp. Hair pieces,
wigs A wig is a head covering made from human or animal hair, or a synthetic imitation thereof. The word is short for "periwig". Wigs may be worn to disguise baldness, to alter the wearer's appearance, or as part of certain professional uniforms. H ...
, hats, and scarves may be used freely.


See also

* Noncicatricial alopecia *
List of cutaneous conditions Many skin conditions affect the human integumentary system—the organ system covering the entire surface of the Human body, body and composed of Human skin, skin, hair, Nail (anatomy), nails, and related muscle and glands. The major function o ...


References


External links


Cicatricial Alopecia Overview
nbsp;- US National Institute of Arthritis and Musculoskeletal and Skin Diseases {{Disorders of skin appendages Conditions of the skin appendages Hair diseases Scarring