Cicatricial Alopecia — Know It All!
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Overview:
Cicatricial Alopecia is also known as Scarring Hair loss or Scarring Alopecia.
Cicatricial alopecia is a group of rare disorders that destroy hair follicles, the part of the skin from which the hair grows. The follicles are replaced with scar tissue, causing permanent hair loss.
Cicatricial alopecia, also referred to as ‘scarring alopecia,’ includes a category of hair loss disorders in which the hair follicle is irreversibly damaged and replaced by fibrous tissue. Hair regeneration is prevented by the degradation of epithelial stem cells in the outer root sheath bulge at the point at which the arrector pili muscle implants.
Cicatricial alopecia also referred to as ‘scarring alopecia,’ includes a category of hair loss disorders in which the hair follicle is irreversibly damaged and replaced by fibrous tissue. Hair regeneration is prevented by the degradation of epithelial stem cells in the outer root sheath bulge at the point at which the arrector pili muscle implants.
What are types of Cicatricial Alopecia or Scarring Alopecia or Scarring Hair Loss disease?
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, the destruction of the hair follicle is incidental to a non-follicle-directed process or external injuries, 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, 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.
As discussed, to be brief, Cicatricial alopecia has two forms:
- In the primary form, immune cells inflame and destroy the hair follicle.
- There are two types of immune cells that can do this: lymphocytes or neutrophils. These immune cells could either work alone or with each other. In the secondary form, the hair follicle is not the direct target. Instead, it is destroyed by another cause, such as a severe burn, infection, radiation, or a tumor. This content focuses on the primary form. Forms of Cicatricial Alopecias Those involving mostly lymphocytes:
- Lichen planopilaris (LPP).
- Frontal fibrosing alopecia.
- Central centrifugal alopecia.
- Pseudopelade of Brocq. Those involving mostly neutrophils: Folliculitis decalvans. Tufted folliculitis. Those involving both (called mixed inflammatory infiltrate): Dissecting cellulitis. Folliculitis keloidalis
Scalp affected areas can have redness, scaling, increased or reduced pigmentation, pustules, or sinus drainage. Many cases can display little inflammatory signs. The inflammation that kills the follicle is below the surface of the skin and there is generally no “scar” visible on the scalp, but it typically leaves the affected scalp bare and smooth without hair and without the normal markings.
Cicatricial alopecias are further categorized in the active stage of the disease by the type of inflammatory cells that kill the hair follicle. The inflammation may mainly include lymphocytes or neutrophils.
Lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq) are scar alopecias that cause mainly lymphocytic inflammations. Cicatricial alopecias caused by primarily neutrophilic inflammation include decalvan folliculitis and tufted folliculitis. The inflammation also changes from a primarily neutrophilic cycle to a lymphocytic one. Cicatricial alopecias with a combined inflammatory infiltrate include cellulite dissection and keloidalis folliculitis.
Who Develops Cicatricial Alopecia or Scarring Alopecia or Scarring Hair Loss disease?
- Cicatricial alopecias affect healthy men and women of all ages although there is typically no primary scar alopecia in babies. Cicatricial alopecias happen all over the world. There were no epidemiological studies performed to assess the prevalence of cicatricial alopecia. They‘re not popular, in general.
- A few cases of scar alopecia occurring within a family have been recorded. But most patients with scar alopecia do not have a family history of a similar disease. Central centrifugal alopecia most often affects African-born women and can occur in more than one member of the family. Dissecting cellulitis looks like scalp-involving deep cystic acne, and it occurs mainly in dark skinned people. Although more than one type of hair loss disease may be present, non-scarring types of hair loss do not develop into scarring types of hair loss.
What causes Cicatricial Alopecia or Scarring Alopecia or Scarring Hair Loss disease?
The origins of the separate cicatricial alopecias are not well known. Both forms of scar alopecia, however, include inflammation directed at the top of the hair follicle where the stem cells and the sebaceous gland (oil gland) are located. When the stem cells and sebaceous gland are killed, there is no chance of hair follicle regeneration, resulting in irreversible hair loss. Cicatricial alopecia is not contagious.
How to diagnose Cicatricial Alopecia or Scarring Alopecia or Scarring Hair Loss disease?
The first step required for diagnosing cicatricial alopecia is a scalp biopsy. Biopsy results, including the extent of inflammation present, location and amount of inflammation, and other changes in the scalp, are important in order to identify the condition of scar alopecia, to assess the degree of infection, and to choose the correct treatment.
After anesthetizing the local area, the biopsy specimen is taken with a biopsy punch which is an instrument that extracts a skin sample about the size and shape of a small pencil eraser. One or two biopsy specimens are taken and are preferably tested both horizontally and vertically after sectioning the skin samples.
Clinical scalp assessment is also relevant. Itching, burning, pain, or tenderness signs usually signal ongoing activity. Inflammatory symptoms of scalp include redness, scaling, and pustules. For certain active cases, however, there are few symptoms or signs, and the active inflammation is only shown by scalp biopsy. The overall severity and pattern of hair loss are noted, and these help the dermatologist to identify the particular cicatricial alopecia present along with the biopsy findings. A hair-pull examination is carried out to classify areas of active disease where follicles are quickly removed. The pulled hair is placed on a board, and the hair bulbs are examined with a light microscope under low power to determine how many (anagen) hairs are developing and how many (telogen) hairs are resting. Normally, only telogen hairs pull out easily; on the contrary, rising hairs can also pull out easily at sites of active scarring alopecia. Therefore, if there are pustules, cultures may be performed to identify which microbes, if any, may contribute to the inflammation. A detailed evaluation that involves all of these criteria is critical in the diagnosis of cicatricial alopecia and in the identification of characteristics in individual patients that will help with therapy selection.
A dermatologist with a special interest or experience in scalp and hair conditions, who is familiar with current diagnostic methods and therapies, will conduct an evaluation.
Summary
What is the treatment for Cicatricial Alopecia or Scarring Alopecia or Scarring Hair Loss disease?
Medication: if you have cicatricial alopecia, the hair will not grow back, it is necessary to start treatment before too much hair is lost. Treatment depends on what sort of immune cell the hair follicle is killing. Medicines may be taken by mouth, applied to the top of the skin, or injected into areas affected.
However though hair can not grow back after a follicle has been damaged, follicles in the affected area can be stimulated before permanent damage occurs. A medication used to treat high blood pressure will promote hair growth in follicles.
Treatment is typically long in length. This persists until there is control of the symptoms and signs, and hair loss slows or stops. Sadly, even after the symptoms and signs have been eliminated, hair loss will continue silent. It may start again after a number of years after cicatricial alopecia stabilizes, and care will need to be restarted.
- Surgery:
After 1 or 2 years of inactivity of the disease, surgical hair restoration or reduction of the scalp can be useful in returning hair to bald regions. In hair regeneration procedure, also known as hair transplantation or follicular micrografting, follicles are surgically removed from the back of the head and transplanted into bare areas of the scalp. A bald portion of the scalp is extracted in scalp reduction, and the section of the scalp beside it is drawn together to cover the void. It’s important to remember that even after these surgeries cicatricial alopecia can recur.
References:
https://rarediseases.org/rare-diseases/cicatricial-alopecia/
https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3855115/
https://www.niams.nih.gov/health-topics/cicatricial-alopecia
By,
Gopala Krishna Varshith,
Content Developer & Editor,
Clipo.
