Cicatricial Alopecia: Symptoms, Causes and Treatment RichFeel

Cicatricial Alopecia also known as scarring alopecia is an inflammatory hair disorder. It results in permanent hair loss as the hair follicles are irreversibility destroyed due to an inflammation. Hair loss due to Cicatricial Alopecia is seen in about 7% of adult men and women. Some of the hair conditions that lead to scarring of hair follicles and hair loss are Lichen Planopilaris, Central Centrifugal Cicatricial Alopecia and Folliculitis decalvans. Hair loss due to Cicatricial Alopecia can be mentally disturbing as it can lead to permanent baldness.

What is Cicatricial Alopecia or Scarring Alopecia?

Cicatricial Alopecia is primarily characterised by inflammation, progressive hair loss and eventual formation of scar tissue. The scarring occurs to the hair follicle and is not seen on the scalp. The hallmark feature of Cicatricial Alopecia is destruction of follicular Ostia, which often results in irreversible hair loss, which in many cases is permanent. Along with the hair follicles the sebaceous glands are also affected. It affects both adult male and female of all age groups; however it is rarely observed in children. Cicatricial Alopecia is extremely unpredictable; sometimes it progresses at a very slow pace other times it is quick to manifest. It usually starts as small bald patches, which eventually expand over the scalp. Rapid progression is often accompanied by varied symptoms like pain and itching. Cicatricial Alopecia is not a contagious disease and does not spread from person to person.

Initial Classification of Cicatricial Alopecia

Alopecia is primarily classified as scarring (Cicatricial, cicatric meaning the scar) and non- scarring. In scarring alopecia, the hair follicle is destroyed with less chance for re-growth. In non-scarring alopecia, the hair follicle remains intact which provides better chance for re-growth of hair. Cicatricial Alopecia is further classified into primary and secondary types. In either type, there is an inflammation occurring at the hair follicle that causes hair fall and forms a scar.

Primary Cicatricial Alopecia: In primary Cicatricial Alopecia, the hair follicle is essentially targeted by the immune system. This is often an abnormal reaction of the immune system and further evokes an inflammatory response in the hair follicle. Further classification of Primary Cicatricial Alopecia is done on the basis of the presence of different types of inflammatory cells like lymphocytes and neutrophils

Secondary Cicatricial Alopecia: In the secondary type, the hair follicle is destroyed by external physical factors like burns, severe infections, tumours, allergens or radiation.

Scarring Alopecia Terminology Based on Histological Features

There are several diseases that look and present similar clinical manifestations like scarring alopecia. The confirmed diagnosis of Cicatricial Alopecia leads to the establishment of the further course of treatment. Since this disease comprises of a group of disorders, narrowing the diagnosis to the sub-types helps to choose appropriate treatment options and prognosis of the disorder is ruled. Many a time the clinical presentation is non-specific and misguiding, hence accurate examination accompanied by supportive literature is preferred.

Primary Scarring alopecia that is caused to inflammation encompasses a group of disorders that are classified based on the presence of inflammatory cells such as lymphocytes and neutrophils.

Lymphocytic Alopecia: This type is seen in Lichen Planopilaris and it has several variants like Frontal Fibrosing Alopecia, pseudopelade and central centrifugal Cicatricial, which show similar clinical presentations.

• Frontal Fibrosing Alopecia is observed in post menopausal women and it affects the frontal hair line where hair loss and receding hairline is observed.
• Pseudopelade is however idiopathic and an inflammatory disorder with patches of alopecia. It progresses at a slow pace, is asymptomatic and often the alopecia is irreversible.
• Central centrifugal Cicatricial Alopecia is more commonly observed in African- Americans. In this disease the bald patch spreads centrifugally from the crown region. Follicular degeneration along with irreversible hair loss is observed and pathologic findings present inflammation of the follicles. Sometimes Cicatricial Alopecia is observed in patients with a pre-existing systemic disease like Lupus Erythematosus.

Neutrophillic Alopecia: Folliculitis decalvans and tufted folliculitis fall under this category. In Folliculitis decalvans, there is chronic inflammation of the hair follicle that causes alopecia and scarring. This disease affects mainly the scalp but other hair bearing regions can be affected too. The diagnostic and pathologic findings present purulent follicles and follicular destruction accompanied by inflammation. In Tufted folliculitis several hairs emerge out of a single follicle giving it a tooth brush like appearance. The inflammation of this follicle is observed to be due to a Staphylococcus aureus infection. The entire tuft of hair falls off when this follicle is inflamed and affected.

Mixed Alopecia: In some forms of alopecia the inflammation is due to both lymphocytes and neutrophils. Some examples of these disorders are Acne keloidalis and Acne necrotica. Predominantly observed in people of African descent, there is characteristic presence of papules on the neck and scalp of the affected individuals.

Signs & Symptoms of Cicatricial Alopecia

Cicatricial Alopecia may present some symptoms or maybe asymptomatic altogether. Some patients present pain, tightness on the scalp, redness and visible inflammation. The progression of scarring alopecia is unpredicted; it either advances slowly without symptoms or progresses quickly presenting a range of symptoms. If the progression is rapid one might experience certain symptoms like itching and burning sensations, but if the hair loss is fast one might not experience any discomfort. Sometimes purulent pustules are also observed. The affected individuals show smooth bald patches with little or no pigmentation.

Some typical symptoms of Cicatricial Alopecia include,

• Pain in hair roots
• Burning sensation on scalp
• Formation of crusts
• Red blisters
• Smooth bald patches on scalp
• Pigmentation

Causes of Cicatricial Alopecia

The primary underlying cause of Cicatricial Alopecia is not fully understood. There are many forms and variants of scarring alopecia but destruction of the hair follicle is the primary cause. Also, some kind of inflammation is observed in all the cases. This inflammation can involve lymphocytes or neutrophils or both (mixed). This inflammation can be triggered by any particular stimulant; it can be due to a faulty immune response, severe pathogenic or fungal infection, potential allergens, and chemical irritants, aggressive hair styling methods or unusual lipid build-up. An abnormal response of the immune system at the follicle is another suspected cause of inflammation. Scarring alopecia is not a contagious disease. The characteristic feature of this disorder is the destruction of the follicle due to any internal or external stimuli, which further progresses to form of a scar, hence the name Scarring Alopecia. The disorder usually initially starts as small bald patches with later spreads over the scalp in a centrifugal pattern.

Common causes on Cicatricial Alopecia include,

• Destruction of hair follicles due to Inflammation
• Genetic factors
• Changes in lipid metabolism
• External factors such as use of harsh chemicals , excessive hair styling, injury on scalp, burns

Diagnosis of Cicatricial Alopecia

A careful dermatologic examination helps in accurate diagnosis of scarring alopecia. The dermatologist first looks for evidence of destruction of follicular ostia. Furthermore, a skin biopsy is very important to confirm the diagnosis. A delay in diagnosis may cause the disease to advance unchecked. Microscopic investigation of the biopsy sample reveals the type of inflammatory cells present, which helps in differential diagnosis. The symptoms presented by the affected individual are also noted and the pattern of hair loss is observed. Sometimes purulent pustules are observed in the patients, the pus from these pustules is investigated for infections and presence of pathogens. A very recent advancement in the diagnostic technology is trichoscopy. This is a non-invasive method used to examine the scalp and the hair follicle. It makes the diagnostic procedure easier as it is non- invasive and helps detect different types of hair and scalp abnormalities.

Treatment Options of Cicatricial Alopecia

Treatment of Cicatricial Alopecia depends upon the diagnosis and the type of alopecia detected. In scarring alopecia, there is permanent loss of hair, hence the treatment is difficult. Since the disease is classified based on the type of inflammation, the treatment options also vary accordingly.

Treatment of Lymphocytic Alopecia: Inflammation that involves lymphocytes is treated using corticosteroids and anti-inflammatory medications. These medicines are administered orally, topically or intravenously.

Treatment of Neutrophillic Alopecia: In the case of Inflammation due to neutrophils, antibiotics are administered to eliminate the infection causing inflammation. Other drugs like methotrexate, cyclosporine and isotretinoin are also prescribed.

Hair Transplantation as Treatment for Cicatricial Alopecia:

The progression of hair loss in scarring alopecia cannot be predicted. At times it advances at a very slow pace without any symptoms other times it progresses quickly and in a rapid manner. Relapse is often observed and when it occurs, the treatment needs to be repeated. Hair re-growth is difficult because along with the follicle the epithelial stem cells are destroyed which provides little scope for regeneration.

Once the underlying tissue is damaged transplant may or may not help to cover the scalp well since the survival of implanted follicle depends on vascularity of the underlying tissue.

Affected Populations

Cicatricial Alopecia is observed in men and women of all ages, though adults are more likely to be affected. Children are rarely affected. Individuals of African- American ancestry are prone to be affected by Central Centrifugal Cicatricial Alopecia. It is observed that they are genetically predisposed to developing this disorder. Central Centrifugal Cicatricial Alopecia (CCCA) is also called hot comb alopecia because of the common practice of using a hot comb to braid hair in the black population. This hot comb method serves as a physical aggressive insult to the hair follicles and causes inflammation leading to scarring alopecia. The crown is initially affected and the bald spot spreads in a centrifugal pattern. Also, another type of Cicatricial Alopecia called Frontal Fibrosing Alopecia is observed in post-menopausal women. In this type of alopecia inflammation due to lymphocytes is noted and the forehead hairline along with eyebrows is affected.

Related Disorders

There are a few related disorders related to Cicatricial Alopecia, which present symptoms similar to those of scarring alopecia. Differential diagnosis is possible based on certain distinctions among the different types observed. One such example is that of Cutaneous Lupus Erythematosus, which affects the skin causing some pigmentation and scaring. This disease is observed predominantly in females.