What is Alopecia Cicatrisata (Pseudopelade)?

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Alopecia Cicatrisata (Pseudopelade)

Pseudopelade is also known as Alopecia Cicatrisata. Pseudopelade is a very rare condition primarily affecting women and sometimes children. Initially, Pseudopelade causes development of well-defined patches of hair loss which for some people may deteriorate to near total scalp hair loss with individual surviving hairs. The history of Pseudopelade for affected individuals is usually slow, lasting many years. For every male affected, three females get Pseudopelade.

Pseudopelade is a poorly defined condition and frequently it is confused with hair loss caused by Lichen Planopilaris or Lupus Erythematosus. Indeed some claim that Lichen Planopilaris and Pseudopelade are one and the same condition. Diagnosis of Pseudopelade can be quite difficult, but some basic clinical pointers to look for in the early stages include irregular shaped and confluent patches of alopecia and some inflammation (erythema). A skin biopsy can help significantly.

It is not understood how or why Pseudopelade occurs, although some experts suspect that it is another autoimmune-based form of hair loss. Alopecia Areata is widely regarded as an autoimmune-based mechanism of hair loss. However, Pseudopelade is not the same as Alopecia Areata as the inflammation with Pseudopelade is typically around the upper hair follicle whereas the inflammation with Alopecia Areata is primarily around the lower hair follicle bulb region. In addition, while Alopecia Areata can persist indefinitely, Pseudopelade progresses, sometimes over several years, and then stops. The result is patches of hair loss where the hair follicles are generally destroyed. The inflammation in the skin subsides at the same time as the hair loss stops.

Transplantation of hair is used as a treatment for pseudopelade but it is important to ensure that the condition has completed its full course. Most dermatologists look for the hair loss lesion to remain stable for at least 6 months before considering transplantation. Because pseudopelade can involve inflammation and scarring, it is unlikely that a hair transplant would last very long when the hair loss is actively advancing. As you might suspect, the transplanted hair follicles could also come under attack and be destroyed. So it is important to ensure that the pseudopelade has stopped progressing before attempting transplantation. When pseudopelade is active, the only treatment available is use of corticosteroids against the inflammation. Corticosteroids can help when there inflammation is involved but when there is little or no inflammation the corticosteroids have little positive effect.

Diagnosis

Differential Diagnosis

  • Seborrheic dermatitis
  • Atopic dermatitis
  • Psoriasis
  • Alopecia areata
  • Traumatic alopecia: trichotillomania, traction alopecia
  • Pseudopelade
  • Folliculitis decalvans
  • Impetigo
  • Lesions of systemic lupus erythematosus (i.e., discoid lupus)
  • Syphilis
  • Histiocytosis
  • Scleroderma

A scalp biopsy for the diagnosis of cicatricial alopecia is the necessary first step. Findings of the 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.

The biopsy specimen is taken with a biopsy punch, which is an instrument that removes a sample of skin about the size and shape of a small pencil eraser, after anesthetizing the local area. One or two biopsy specimens are taken, and ideally are examined after sectioning the skin samples both horizontally and vertically.

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 active 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, together with the biopsy findings, these enable the dermatologist to diagnose the specific cicatricial alopecia present. A hair pull test is performed to identify areas of active disease in which follicles are easily pulled out.

The pulled hairs are mounted on a slide and the hair bulbs are viewed under low power with a light microscope to determine how many are growing (anagen) hairs and how many are resting (telogen) hairs. Normally, only telogen hairs pull out easily; in contrast, in sites of active scarring alopecia, growing hairs may also pull out easily. In addition, if pustules are present, cultures may be performed 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.

Evaluation should be done by a dermatologist with a special interest or expertise in scalp and hair disorders, and who is familiar with current diagnostic methods and therapies.

Workup

The clinical presentation is fairly distinctive, but other diagnoses can mimic tinea capitis, and confirmation of the diagnosis is generally advised.

Wood’s lamp examination is generally not helpful because T. tonsurans and T. violaceum do not fluoresce.

Hairs infected by M. audouinii and M. canis produce a brilliant green fluorescence.

Laboratory Tests

Potassium hydroxide (KOH) preparations can be useful for immediate confirmation of infection, but the sensitivity and specificity depend on the experience of the individual performing the test and the morphology of the lesion scraped.

  • False‐negative and false‐positive results occur.
  • Culture is recommended.

To obtain a fungal culture, rub several areas of the scalp with a clean, disposable toothbrush or sterile cotton swab, and inoculate onto an antibiotic‐enriched mycologic media.

Initial classification

The initial determination of alopecia type usually begins with the establishment of alopecia as either a scarring (cicatricial) alopecia or nonscarring alopecia. Nonscarring alopecias tend to have preserved follicular ostia. No clinically visible inflammation is noted in most presentations, although histologic inflammation may be present. The most common nonscarring alopecias include alopecia areata and telogen effluvium.

Scarring alopecias have loss of follicular ostia, or atrophy. Clinical inflammation is frequently, but not always, present. Histologic inflammation may be present. Ultimately, histologic confirmation is the best method to confirm the presence of a fibrosing/scarring process with loss of hair follicles.

Many alopecia types are biphasic. For example, androgenetic alopecia eventually results in loss of ostia and thus may appear like a scarring alopecia. This article focuses on the alopecia types that are believed to be due to an inflammatory response with rapid secondary scarring if not controlled.

Scarring alopecia terminology based on clinical features

Once the patient is determined to have scarring alopecia, establishing and clarifying the diagnostic options and terminology is important to assist in confirming the diagnosis, initiating treatment, and suggesting a prognosis. Several manuscripts have examined and attempted to clarify the literature findings as summarized below.

Central centrifugal cicatricial alopecia (CCCA)

CCCA is a diagnostic category adopted by the North American Hair Research Society to encompass terms such as hot comb alopecia, follicular degeneration syndrome, pseudopelade in African Americans, and central elliptical pseudopelade in whites.  Despite the many attempts to clarify and unify the terminology of central CCCA patterns of scarring alopecia, CCCA is not clearly a diagnostic entity in and of itself.

Pseudopelade

Pseudopelade is a term used to describe a clinically noninflammatory patchy alopecia. The most common condition to produce this appearance is lichen planopilaris. The term “idiopathic pseudopelade” refers to a distinct fibrosing alopecia characterized by a thin dermis, with dense eosinophilic dermal collagen and thick recoiled elastic fibers. The fibrous tracts are broad and may contain granulomas, but the surrounding elastic sheath is preserved.

Traction alopecia

As the name implies, traction alopecia is alopecia secondary to physical traction. Variant presentations exist with terminology that is optional for differentiating purposes. This includes alopecia linearis frontalis (ALF), more commonly known as marginal alopecia, and chignon alopecia, which is when a tight hair bun causes the traction changes.

Secondary systemic scarring alopecia

Secondary systemic scarring alopecia is a term used when the alopecia is a feature of a systemic disease (eg, scleroderma, discoid lupus erythematosus). Discoid lupus erythematosus is often a cause of alopecia. In the classic lupus classifications scheme, discoid lupus erythematosus is one of the chronic cutaneous lupus types (along with tumid lupus and lupus panniculitis).

Trichotillomania

Trichotillomania is a nonscarring psychiatric alopecia that can have a scarring clinical presentation. Trichotillosis is an alternative term that some physicians believe is more acceptable to patients.

Signs & Symptoms

Affected areas of the scalp may have redness, scaling, increased or decreased pigmentation, pustules, or draining sinuses. Other cases may show little signs of inflammation. The inflammation that destroys the follicle is below the skin surface and there is usually no “scar” seen on the scalp, although the affected scalp is usually left bare and smooth without hair and without the usual pore markings.

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 involve predominantly lymphocytes or neutrophils.

Cicatricial alopecias that involve predominantly lymphocytic inflammation include lichen planopilaris, frontal fibrosing alopecia, central centrifugal alopecia, and pseudopelade (Brocq). Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans and tufted folliculitis. Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. Cicatricial alopecias with a mixed inflammatory infiltrate include dissecting cellulitis and folliculitis keloidalis.

Causes

The cause of the various cicatricial alopecias is not well understood. However, all types of cicatricial alopecias involve inflammation directed at the upper part of the hair follicle where the stem cells and sebaceous gland (oil gland) are located. If the stem cells and sebaceous gland are destroyed, there is no possibility for regeneration of the hair follicle, leading to permanent hair loss. Cicatricial alopecias are not contagious.

Affected Populations

Cicatricial alopecias affect healthy men and women of all ages, although primary cicatricial alopecia is not usually seen in children. Cicatricial alopecias occur worldwide. Epidemiologic studies have not been performed to determine the incidence of cicatricial alopecias. In general, they are not common.

There have been a few reports of cicatricial alopecia occurring in a family. However, the majority of patients with cicatricial alopecia have no family history of a similar condition. Central centrifugal alopecia most commonly affects women of African ancestry and may occur in more than one family member. Dissecting cellulitis looks like deep cystic acne involving the scalp, and it occurs primarily in dark-skinned men. 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.

Related Disorders

Symptoms of the following disorder can be similar to those of cicatricial alopecia. Comparisons may be useful for a differential diagnosis:

Chronic cutaneous lupus erythematosus (CCLE) is predominantly a cutaneous disease with few systemic complications. Scarring and disfiguring changes in the skin (discoid lesons) are commonly seen in this disease. It occurs more frequently in females than males and more commonly in adults than children.

Standard Therapies

Primary cicatricial alopecias are classified by the predominant type of inflammatory cells that attack the hair follicles (i.e., lymphocytes, neutrophils, or mixed inflammatory cells), and treatment strategies are different for each subtype and each patient.

Treatment of the lymphocytic group of cicatricial alopecias including lichen planopilaris, 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, doxycycline, mycophenolate mofetil, cyclosporine, or pioglitazone. Topical medications may include corticosteroids, topical tacrolimus, topical pimecrolimus, or Derma-Smoothe/FS scalp oil. Triamcinolone acetonide (a corticosteroid) may be injected into inflamed, symptomatic areas of the scalp.

Treatment of the neutrophilic group of cicatricial alopecias (folliculitis decalvans, tufted folliculitis) is directed at eliminating the predominant microbes that are invariably involved in the inflammatory process. Oral antibiotics are the mainstay of therapy. Topical antibiotics and anti-inflammatory medications may be used to supplement the oral antibiotics.

Treatment of the mixed group of cicatricial alopecias (dissecting cellulitis, folliculitis keloidalis) may include antimicrobials, anti-inflammatory medications, isotretinoin (starting dose must be small). Infliximab may be helpful in treatment-resistant dissecting cellulitis.

The course of cicatricial alopecia is usually prolonged. Treatment is continued until the symptoms and signs of scalp inflammation are decreased, and progression of the condition has been controlled. Itching, burning, pain, and tenderness, and scalp redness, scaling, and/or pustules, can usually be controlled by current treatments. Unfortunately, the progression of the hair loss may continue silently even when the symptoms and signs are cleared. Cicatricial alopecia may reactivate after a quiet period and treatment may have to be repeated.

Surgical treatment for cosmetic benefit is an option in some affected individuals 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 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 the above treatment early to control the inflammatory process. In addition, minoxidil solution or foam (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 minimal, and in other cases progression can be rapid and extensive. Usually there is sufficient hair remaining to cover the affected scalp areas; relatively few patients require a hair piece.

Hair care products and shampoos are generally safe as long as they are non-irritating to the scalp. A dermatologist can recommend specific shampoos and products to decrease scalp symptoms, scaling and inflammation and will recommend frequency of their use. Hair pieces, wigs, hats, and scarves are all safe, will not aggravate your condition, and may be used freely.

General Discussion

Cicatricial alopecia usually includes a range of rare disorders related to the destruction of the hair follicle and gets replaced by scar tissue which may lead to permanent hair loss. Typically the hair loss is gradual, without symptoms, and is unnoticed for long periods. In some the hair loss is also associated with rapidly progressive symptoms such as itching, burning and pain. The inflammation that destroys the follicle is located below the surface of the skin and there is usually no “scar” seen on the scalp. Affected areas of the scalp may show little signs of inflammation.

There are two known types of cicatricial alopecias classified as primary Cicatricial alopecias or secondary Cicatricial alopecias. For primary cicatricial alopecias in the hair follicle is the target of the destructive inflammatory process. In secondary cicatricial alopecias, destruction of the hair follicle is an “accidental” non-follicle-directed process or external injury, such as severe infections, burns, radiation, or tumors.

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 lymphocytes or neutrophils. Cicatricial alopecias that predominantly involve lymphocytic inflammation include lichen planopilaris, frontal fibrosing alopecia, chronic cutaneous lupus erythematosus, central centrifugal alopecia, pseudopelade (Brocq), alopecia mucinosa, and keratosis follicularis spinulosa decalvans.

Cicatricial alopecias that are due to predominantly neutrophilic inflammation include folliculitis decalvans and dissecting cellulitis.

Sometimes the inflammation shifts from a predominantly neutrophilic process to a lymphocytic process. Cicatricial alopecias with a mixed inflammatory infiltrate include folliculitis keloidalis and erosive pustular dermatosis.

Prognosis – Alopecia areata

In most cases which begin with a small number of patches of hair loss, hair grows back after a few months to a year. In cases with a greater number of patches, hair can either grow back or progress to AA totalis or, in rare cases, AA universalis.

Effects of AA are mainly psychological (loss of self-image due to hair loss). Loss of hair also means the scalp burns more easily in the sun. Patients may also have aberrant nail formation because keratin forms both hair and nails.

Hair may grow back and then fall out again later. This may not indicate a recurrence of the condition, however, but rather a natural cycle of growth-and-shedding from a relatively synchronised start; such a pattern will fade over time. Episodes of AA before puberty predispose one to chronic recurrence of the condition.

Alopecia can certainly be the cause of psychological stress. Because hair loss can lead to significant appearance changes, individuals may experience social phobia, anxiety, and depression.

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