Scarring (Cicatricial) Alopecia
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“Cicatricial alopecia” or scarring alopecia is referred to as a diverse group of rare disorders that damage the hair follicle, destroys associated sebaceous (oil) gland, replace it with scar tissue, and results in permanent hair loss. The clinical course of this phenomenon is highly unpredictable and variable.
Hair loss through Cicatricial alopecia may be slowly progressive over few years, without any symptoms, and unobserved for long periods. Or the hair loss may be speedily devastating within months and associated with severe pain, inflammation and itching. The burning that damages the follicle is beneath the surface of the skin and there is generally no “scar” vissible on the scalp.
Affected areas of the scalp may display some signs of increased or decreased pigmentation, red rashes, burning sensation, scaling, pustules or draining sinuses. Cicatricial alopecia occurs in otherwise healthy women and men of all ages, is not transferrable, not inherent and is observed worldwide.
The cause of the various cicatricial alopecias is not well understood. What is known so far is that swelling, heat, pain/discomfort, or redness, directed at usually the upper part of the hair follicle, where the sebaceous glands (oil gland) and stem cells are located. Stem cells are cells that can develop into different kinds of cells. If the sebaceous glands and stem cells are destroyed, the hair follicle would fail to regrow, and hair is permanently lost.
A Research indicated that a loss of function of a “master regulator” known as the peroxisome proliferator-activated receptor gamma, or PPAR gamma can be a cause of Cicatricial alopecia. PPAR gamma plays a vital role in the conservation of hair follicle cells, including sebaceous glands and stem cells. Decreased PPAR gamma results in sebaceous gland dysfunction, which causes abnormal buildup of “toxic” lipids. This abnormal buildup of lipids stimulates burning that eventually destroys the hair follicle.
Who is affected?
Cicatricial alopecia occurs in both men and women of all ages and could rarely cause children too. Some afflictions may demonstrate in the late teenage years. There have been a very few incidence reports of cicatricial alopecia occurring in a family. Though, the large number of patients suffering with cicatricial alopecia has no family history of the same condition.
A particular type of cicatricial alopecia known as “Central centrifugal cicatricial alopecia” typically affects women of African ancestry and may occur in most family members. Frontal fibrosing alopecia is most commonly observed in post-menopausal women but also appears in young men and women. While it is possible to experience different type of hair loss condition at one time, non-scarring forms of hair loss do not convert into scarring types of hair loss.
While a telltale sign of cicatricial alopecia is the non-existence of the openings of the hair follicles through which the hair fibers make an appearance from the skin, a scalp biopsy is quite important to confirm the diagnosis. When conducting a biopsy, it is essential to choose an area that still has some burning, as it is complicated to distinguish the different types of cicatricial alopecia in late-stage illness.
Also it is very important to mark that analysis alone cannot be the final step; the literature is packed with examples of diagnostic mimics in alopecia. This includes patients presenting with no inflammatory, non-scarring, alopecia that is afterwards found to be inflammatory and scarring. Much of the ambiguity is often based in the similarities of clinical presentation, which focuses again on the importance of not basing diagnosis completely on clinical examination findings alone.
A hair-pull test is useful in aiding the diagnosis and directing the doctor where to biopsy. In a hair pull test, the surgeon will clasp onto a bunch of hair in an area of active disease. The result of this test depends upon the amount of hairs removed with each single pull and by noticing if the hairs still have intact follicles. A positive pull test will usually produce 5 or more hairs. It suggests active inflammation that is destroying and weakening the follicles around the surrounding tissue.
The kind of inflammation observed on the scalp biopsy together with the overall pattern of the hair loss supports the physician to identify a particular type of cicatricial alopecia and its level of activity. This helps the doctor to choose the most appropriate therapy.
Other factors that help the doctor make a diagnosis and assist-management include the following:
- The date of commencement/aggression and degree of progression
- Prior hair loss cosmetic treatments or hair treatments
- Family history – cicatricial alopecia merely presents itself in family members; except central centrifugal alopecia
- Past medical history – any trauma, illness, mental stress or infections.
- Symptoms of inflammation, acute pain or itching.
- Use of Medications like androgens or exogenous
- Any hair regrowth – this would head towards a dubious diagnosis of cicatricial alopecia
In addition, if pustules are found, the dermatologist may use cultures to determine which microbes, if any, may be giving rise to the inflammation. However, as evaluated, often there are few signs or symptoms and only the scalp biopsy exhibits the active inflammation. Ultimately, the overall severity and pattern of hair loss for further comparison can be illustrated with cicatricial alopecia images.
Lichen Planopilaris (LPP)
Lichen planopilaris (LPP) is a form of scarring alopecia that mainly occurs in adulthood, specifically in women. Patients usually have a history of patchy baldness with severe itching, inflammation and painful discomfort. The phenomenon may be changing and may be observed as a steady progression of patchy hair loss or induced hair thinning over many years, or a rapid progression over few months. LPP is usually regarded as a scalp hair demonstration of a disease process called as lichen planus. With lichen planus, there may be nail, skin, or mucous membrane association.
On analysis of an individual with suspected LPP, the absence of follicular ostia (the openings of the hair follicles by which the hairs makes it way from the skin) in the scalp is an active signal that this course is due to a scarring type of hair loss, and not because of hereditary hair loss. In LPP, there may be signs of scalp irritation and redness around the margins of the regions of hair loss, however, the middle part of the bare regions is smooth. There may be single or multiple abrasions found anywhere on the scalp. If LPP is suspected, a scalp biopsy will be conducted to assure the diagnosis.
The treatment is majorly suggested for patients with active disease progression. Various scalp Therapies are objected at preventing disease progression. In specific terms, medications including mycophenolate mofetil and hydroxychloroquine that are effective in minimizing symptoms and signs of LPP are indicated to patients. Even with dissolution of the symptoms, there still may be progression of the hair loss in lichen planopilaris.
The diagnosis of lichen planopilaris (LPP) is confirmed by a composition of histological and clinical features.
Lichen planopilaris (LPP)
Frontal Fibrosing Alopecia (FFA)
Frontal fibrosing alopecia (FFA) is a scarring type of hair loss largely seen in females. A common disorder is recession of the hairline or highly visible temples, often related with loss of eyebrows. In disparity to usual baldness (female pattern alopecia) where there is actual hair thinning, in FFA there are full thickness hairs diffused with localized regions of balding. Often times, there is noticed a recession of the frontal hairline of about 1 inch associated with redness, itching or irritation in the scalp, particularly around the hair shafts. There commonly is itching linked with this condition.
This is a prospective clinical variant of lichen planopilaris, with almost alike histologic characteristics. Frontal fibrosing alopecia has been linked with the postmenopausal state- postmenopausal frontal fibrosing alopecia, though not all individuals with this kind of diagnosis are postmenopausal.
The skin along the receding hairline is usually pale and seems like a band that may advance continually behind and above the ears. The hair recession could either be slow or fast or – and at times it is self-limiting too. The loss of eyebrows, which generally occurs simultaneously as the hairline recession (but may precede the hairline recession by several years), can be partial or overall. In some cases, patients may also complain about loss of eyelashes, or loss of hair on other body parts (legs or arms). These are all clinical symbols supporting a diagnosis of FFA.
Following a physical examination and an initial history, the hair expert will normally perform a scalp biopsy. If a biopsy puts a stamp of the diagnosis of FFA, the very next step is to examine the treatment plans. The prime most Treatment strategy for this condition is:-
- Medical treatment with oral medications inclusive of doxycycline, intralesional injection of steroids, hydroxychloroquine and topical such as corticosteroids.
- For loss of eyebrows, intralesional corticosteroids are taken and repeated in 1.5 months to 3 months intervals. This may lead to reversal of the hair loss in patients who still have few remaining brows.
Central Centrifugal Cicatricial Alopecia (CCCA)
Central Centrifugal Cicatricial Alopecia (CCCA) is a kind of scarring alopecia that predominantly occurs in black women and results in permanent hair loss. However, it may appear in men and among individuals of all origins and hair colour (though barely). Middle-aged females are most commonly affected.
The exact cause of CCCA is not well understood and is likely multifactorial. Hair practices including tight extensions or weaves, hair-stylers, hot comb and relaxers have been suspected for ages. Other proposed causes include autoimmune disease, bacterial/fungal infections and inheritance. Diabetes mellitus is one such medical condition of type 2 situation.
Hair loss in central centrifugal cicatricial alopecia suspected patients typically starts at the vertex or mid-scalp and stretches outward in a centrifugal pattern. There is a significant loss of the follicular openings on evaluation of the scalp. Thus, the scalp may visibly look shiny. Generally there is mild tenderness, itch and inflammation in the affected area, while some people do not show any symptoms at all. Hair breakage may be an early signal of CCCA.
Early diagnosis of CCCA is quite essential as medical intervention can prevent any further progression that often leads to severe, permanent hair loss. Diagnosis usually depends upon scalp biopsy, clinical characteristics, and other hair loss disorders. Scalp biopsy should be performed from an edgy patch of alopecia rather than center of a scarred region. Premature peeling of the inner root sheath is a common characteristic.
The goal is to put a pause on progression of disease and prevent further hair loss. In parts of scalp where the follicles have been replaced with fibrosis, re-growth is impossible.
Treatment for CCCA includes
Anti-inflammatory agents such as:
- Intralesional steriods or topical steroids (eg. Clobetasol)
- Tetracyclines (eg: doxycycline 100 mg twice daily, taken for few weeks to months)
- Calcineurin inhibitors: pimecrolimus cream, tacrolimus ointment
- topical tacrolimus
- topical pimecrolimus
- FS scalp oil.
- A corticosteroid ( Triamcinolone acetonide) may be infused into burned, symptomatic parts of the scalp.
Hair restoration can be taken into account in patients with well-controlled CCCA for minimum one year. Also discontinuing of traumatic hair care practices is an important aspect of CCCA treatment.
It is important to notice that, hair will not grow back once the follicle is destroyed. However, it is possible to treat the inflammation in and around the surrounding follicles until they are completely damaged. For this reason it becomes essential to start with the appropriate treatment as early as possible to halt the inflammatory process.
Minoxidil solution (5%) applied once or (2%) twice daily to the scalp may aid to stimulate any remaining, tiny and unscarred follicles. The rate of progression of hair loss is unforeseeable. In few cases, progression is slow n steady and there is enough hair remaining on the scalp to cover the affected portions or bald patches; while in other cases, progression can be rapid and enormous.
If you feel that you may have symptoms of a scarring alopecia or other types of hair loss, it is crucial to visit an expert hair loss specialist as soon as possible.