Alopecia Cicatrisata: Causes, Symptom and Treatment

Alopecia Cicatrisata Summary

Summary

  1. Alopecia cicatrisata is a scarring hair loss disorder caused by follicular inflammation and destruction.
  2. Common symptoms include patchy hair loss, redness, pustules, and scaling on the scalp.
  3. Treatment focuses on reducing inflammation with medications like corticosteroids and immunosuppressants.

Introduction

Alopecia cicatrisata, or scarring alopecia, is a rare hair loss condition that permanently damages hair follicles, resulting in irreversible hair loss.

Unlike other forms of alopecia, this type involves inflammation that harms hair follicles, leading to scar tissue formation and preventing hair from growing back.

Scarring alopecia can impact anyone regardless of age or gender, and while it can appear suddenly or develop gradually, it often leads to significant emotional distress due to its lasting effects.

There are two primary kinds of scarring alopecia: primary cicatricial alopecia, which targets hair follicles directly with inflammation, and secondary cicatricial alopecia, where the follicles are damaged by other skin conditions such as burns, infections, or tumors.

Early recognition and treatment of scarring alopecia is crucial to stop progression since scarred hair follicles cannot recover.

Causes

The causes of alopecia cicatrisata are varied and involve genetic, autoimmune, and environmental elements. The complexity of the condition makes it hard to pinpoint a single cause.

  • Autoimmune Response: Many scarring alopecia types stem from an autoimmune process where the body incorrectly attacks hair follicles as if they were harmful. This is typical in disorders like lichen planopilaris (LPP) and discoid lupus erythematosus (DLE).
  • Genetic Predisposition: There may be a genetic risk factor that heightens the chance of developing scarring alopecia, especially in autoimmune conditions. However, clear genetic mutations linked to this type of alopecia are not yet established.
  • Inflammatory Skin Disorders: Ongoing inflammatory skin issues, such as psoriasis or eczema, can lead to secondary scarring alopecia by damaging hair follicles, resulting in scars.
  • Infections: Some bacterial and fungal infections can cause folliculitis or abscesses, which, if untreated, may lead to scarring and permanent hair loss.
  • Trauma or Burns: Physical damage to the scalp from burns, chemicals, or radiation therapy can trigger secondary scarring alopecia.

Types of Primary Scarring Alopecia

Scarring alopecia can be further categorized based on histological patterns and affected cells:

  • Lichen Planopilaris (LPP): An inflammatory condition often found in women, marked by redness, itching, and scaling around hair follicles.
  • Discoid Lupus Erythematosus (DLE): A type of lupus affecting the skin, leading to red, scaly patches primarily on the scalp, causing scarring and hair loss.
  • Central Centrifugal Cicatricial Alopecia (CCCA): Common among African American women, this type starts in the center of the scalp and extends outward.
  • Frontal Fibrosing Alopecia (FFA): A variation of LPP, more frequent in postmenopausal women, characterized by hair loss at the front hairline and sometimes the eyebrows.

Symptoms

Symptoms of alopecia cicatrisata vary by type but generally include localized or widespread hair loss with signs of inflammation on the scalp. Common symptoms are:

  • Hair Loss: Gradual or sudden loss of hair, often in patches, with areas where hair fails to regrow.
  • Redness and Scaling: The scalp may look red and inflamed, with patched areas that are scaly or rough around affected follicles.
  • Pain or Burning Sensation: Some individuals may feel discomfort, including pain, itching, or a burning sensation in the affected areas.
  • Permanent Scarring: As the disease worsens, hair follicles are destroyed and replaced by scar tissue, leaving smooth, shiny, bald patches.

In severe or rapidly advancing scarring alopecia, symptoms may be extensive, impacting large scalp areas and resulting in major cosmetic issues.

Risk Factors

Several risks may elevate the chances of developing scarring alopecia:

  • Autoimmune Disorders: Individuals with autoimmune diseases, like lupus, rheumatoid arthritis, or vitiligo, Individuals have a higher chance of getting types of scarring alopecia linked to an autoimmune response.
  • Genetics: If someone has family members with autoimmune or inflammatory skin issues, they might be more likely to develop scarring alopecia.
  • Ethnicity: Central centrifugal cicatricial alopecia (CCCA) happens more in African American women, indicating a potential genetic or cultural link.
  • Age and Gender: Certain scarring alopecia forms, like frontal fibrosing alopecia, are more often seen in women after menopause, which suggests hormonal factors might play a role.
  • Environmental Exposures: Long-term exposure to specific chemicals, hair treatments, or tight hairstyles could lead to certain types of scarring alopecia.

Differential Diagnosis

Scarring alopecia can look like other hair loss conditions, so it is important to conduct a thorough differential diagnosis to eliminate similar issues:

  • Alopecia Areata: This autoimmune condition causes sudden patchy hair loss but does not involve scarring or follicle damage.
  • Telogen Effluvium: This is a temporary hair loss type triggered by stress or hormonal shifts, with more generalized hair shedding instead of patchy loss.
  • Androgenetic Alopecia: Known as male or female pattern baldness, this causes gradual thinning rather than patchy hair loss and inflammation.
  • Seborrheic Dermatitis: This chronic skin problem causes redness and scaling on the scalp, but usually does not lead to hair loss or scarring.
  • Folliculitis Decalvans: This rare condition leads to inflammation and damage to hair follicles, often presenting with pustules and crusting on the scalp.
Comparison Table
Differential Diagnosis Definition Symptoms Treatment
Alopecia Cicatrisata A scarring type of hair loss caused by inflammation that destroys hair follicles. Patchy hair loss with scarring, often accompanied by redness, scaling, and pustules. Anti-inflammatory medications, immunosuppressive agents, and sometimes surgical intervention.
Alopecia Areata An autoimmune condition causing sudden patchy hair loss without scarring. Well-defined round patches of hair loss; no redness or scarring. Topical corticosteroids, immunotherapy, and in some cases systemic treatments.
Telogen Effluvium Temporary hair loss caused by stress or hormonal changes. Diffuse hair shedding across the scalp. Resolving the underlying cause; supportive treatments like minoxidil.
Androgenetic Alopecia A genetic condition causing gradual hair thinning over time. Thinning hair in predictable patterns; no inflammation or scarring. Topical minoxidil, oral finasteride, or hair transplantation.
Seborrheic Dermatitis A chronic condition causing redness and scaling on the scalp. Itchy, flaky scalp; no significant hair loss or scarring. Medicated shampoos (e.g., ketoconazole) and topical anti-inflammatory treatments.
Folliculitis Decalvans A rare condition causing follicular inflammation and scarring hair loss. Scalp pustules, crusting, and scarring with hair loss. Antibiotics, anti-inflammatory agents, and sometimes isotretinoin.

Investigation

To diagnose alopecia cicatrisata, it is important to gather a detailed patient history, perform a clinical examination, and conduct further tests to identify the type of scarring alopecia and distinguish it from other hair loss forms.

  • Clinical Examination: Look at the scalp for hair loss patterns, signs of inflammation, redness, and scarring. Dermoscopy can help closely inspect the scalp.
  • Scalp Biopsy: Generally, a biopsy is necessary to confirm scarring alopecia. It shows the type of inflammatory cells present, such as lymphocytes in LPP or neutrophils in folliculitis decalvans, aiding in subtype identification.
  • Histopathological Examination: The biopsy’s histology will display hair follicle destruction replaced by scar tissue and show different inflammatory cell patterns based on the alopecia type.
  • Blood Tests: Tests like antinuclear antibody (ANA) testing can help check for underlying autoimmune diseases like lupus, which could cause scarring alopecia.
  • Trichoscopy: This non-invasive method uses a magnifying camera to check the scalp and hair follicles, spotting features like redness around hair follicles or scaling that assist in diagnosis.

Treatment

Currently, there is no cure for scarring alopecia, and treatment aims at managing inflammation to prevent more hair loss. Early treatment is important to stop further progress, as hair loss may be permanent after follicles are scarred.

  • Topical Corticosteroids: Strong topical corticosteroids help reduce inflammation in mild cases. They are applied directly to affected areas to slow the progress of the disease.
  • Intralesional Corticosteroid Injections: Injecting corticosteroids right into scalp lesions can effectively treat localized inflammation, especially in conditions such as LPP and DLE.
  • Oral Corticosteroids: For more severe or fast-progressing cases, systemic corticosteroids might be prescribed to manage inflammation, although long-term use is not preferred due to possible side effects.
  • Immunosuppressive Agents: Medications like methotrexate, cyclosporine, or mycophenolate mofetil may be useful in severe scarring alopecia cases, particularly if it is linked to an autoimmune response. These drugs lower immune response and cut down inflammation.
  • Hydroxychloroquine: This medicine is often for autoimmune diseases. It helps with inflammatory scarring alopecias like DLE and LPP.
  • Antibiotics: In cases like folliculitis decalvans or with secondary infections, antibiotics such as doxycycline or clindamycin may help decrease bacteria and inflammation.
  • JAK Inhibitors: Janus kinase (JAK) inhibitors are a new group of immune-modulating drugs. They have shown potential for some types of hair loss caused by autoimmune issues, but their use for scarring alopecia is still in research.
  • Surgical Options: After the disease is inactive, hair restoration surgery or scalp micropigmentation may be options to improve appearance. Surgery is only safe if the disease is well-managed and inflammation is not active.
  • Psychological Support and Counseling: Scarring alopecia can be hard to cope with because it is permanent and progressive. Counseling and group support can assist patients in dealing with the emotional effects.

Conclusion

Alopecia cicatrisata, known as scarring alopecia, is a rare and complicated form of hair loss that has lasting effects. Early detection and strong treatment of inflammation are vital to prevent more damage and permanent hair loss.

Although treatment cannot bring back hair follicles once scarred, it can manage inflammation and slow down the disease. Knowing the types, causes, and treatment choices for scarring alopecia enables better management and results for those affected.

Because this condition has a strong emotional and psychological toll, supportive care is very important to improve quality of life and mental health.

References Toggle
  1. Olsen EA, Bergfeld WF, Cotsarelis G, Price VH, Shapiro J, Sinclair R, et al. “Summary of North American Hair Research Society (NAHRS)-sponsored workshop on cicatricial alopecia.” Journal of the American Academy of Dermatology. 2003;48(1):103-110.
  2. Harries MJ, Paus R. “Scarring alopecia.” Current Problems in Dermatology. 2015;47:143-149.
  3. Miteva M, Tosti A. “Pathologic diagnosis of cicatricial alopecia: a review.” Journal of Clinical and Experimental Dermatology Research. 2011;2(4):142.
  4. National Alopecia Areata Foundation. “Understanding Scarring Alopecia.” Available from: https://www.naaf.org
  5. Rajabi F, Drake LA, Senna MM. “Central centrifugal cicatricial alopecia: An approach to diagnosis and management.” International Journal of Dermatology. 2021;60(9):1091-1097.
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