Scarring Alopecia

Scarring alopecia, also known as cicatricial alopecia, comprises a large group of heterogeneous disorders. They can be divided into inflammatory and noninflammatory categories based on their underlying pathogenesis. The inflammatory scarring alopecias can be further grouped into either infectious (see Chapter 9: Hair and Nail Disorders) or noninfectious.
This section will focus on the scarring alopecias that are caused by inflammatory noninfectious processes: chronic cutaneous lupus erythematosus, lichen planopilaris, central centrifugal cicatricial alopecia, cutaneous sarcoidosis, folliculitis decalvans, pseudofolliculitis barbae, and acne nuchae keloidalis.
Scarring alopecias affect all ethnic groups and races; however, certain types of scarring alopecias are more prevalent in African-American and Afro-Caribbean women.

In scarring alopecia, perifollicular inflammation (from autoimmune phenomena, superantigen response of cytokines, or infection) leads to the replacement of the hair follicle by scar tissue resulting in permanent loss of the hair follicle.
In addition direct trauma to the hair follicle from burns, radiation dermatitis, cutaneous malignancies, cutaneous sarcoidosis, or certain hairstyling practices can result in damage and permanent destruction of the hair follicles.

In its early stages, there is no obvious hair loss or scarring noted and the diagnosis is often missed. The patient may present with scaling and itching and is often diagnosed as having “excessive dandruff” or “seborrheic dermatitis.” The patient may then be advised to shampoo more often with an antidandruff shampoo which often results in exacerbation of symptoms.
As the disease progresses, the hair loss becomes more apparent. The loss of follicular orifices (ostia or pores) is a key feature of scarring alopecia (Fig. 19.14), differentiating it from alopecia areata, a nonscarring inflammatory alopecia in which the follicular orifices remain intact.
The process can further evolve and coalesce into larger areas.
The skin within the areas of hair loss may have a thin, shiny, atrophic appearance and may spread centrifugally, with an area of central scarring surrounded by an expanding periphery of erythema.

Chronic Cutaneous Lupus Erythematosus (see also Chapter 34: Cutaneous Manifestations of Systemic Disease)

Basics

Chronic cutaneous lupus erythematosus (CCLE) accounts for about one-third of cases of cicatricial alopecia. It occurs more frequently in African-American women.
Evidence of systemic lupus erythematosus (SLE) or other cutaneous signs of lupus may or may not be present.
Discoid lupus erythematosus—so-named for its discoid, or disk-shaped, lesions—is by far the most common form of CCLE.
SLE, unlike CCLE, may lead to telogen effluvium, which typically presents as a diffuse nonscarring type of hair loss (see earlier discussion); occasionally, the scarring alopecia of CCLE may also be seen in a patient with SLE.

Clinical Manifestations

Typically, the patient with CCLE initially presents with patches of alopecia on the scalp that are red, atrophic, and with mottled pigmentation (areas of hypo- and hyperpigmentation) (Fig. 19.15A,B).
Lesions may be quite pruritic.
Other similar lesions may be observed on the conchae of the external ears or elsewhere on the body (see detail in Fig. 19.15B).

Diagnosis

A scalp biopsy and direct immunofluorescence helps to make the diagnosis.
Serologic studies can be useful to rule out SLE; however, the vast majority of CCLE cases may be isolated phenomena and do not demonstrate the presence of antinuclear antibodies.

Differential Diagnosis

Other causes of scarring alopecias: central centrifugal cicatricial alopecia (see below), tinea capitis, cutaneous sarcoidosis, and traction alopecia.

Management

Superpotent topical corticosteroids or intralesional corticosteroid injections are the first-line therapy for CCLE.
In more extensive or recalcitrant cases, hydroxychloroquine (200 to 400 mg/day) is often effective.
Other therapies that have been used include dapsone, isotretinoin, and thalidomide. The goal of treatment is to alleviate scalp pruritus or discomfort, decrease inflammation, and prevent further destruction of the hair follicles.

Helpful Hints

When faced with a scarring alopecia in a woman of color, it is very important to rule out cutaneous sarcoidosis or, less commonly, a fungal infection of the scalp (tinea capitis), both of which can lead to a scarring alopecia.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency screening is required prior to treatment with dapsone because patients with G6PD deficiency are more prone to the hematologic side effects of this drug.
Patients taking hydroxychloroquine need to be monitored for anemia and require retinal examination prior to and during treatment because of the potential for retinopathy.

Lichen Planopilaris

Basics

Lichen planopilaris (LPP) accounts for approximately another third of cases of scarring alopecia and is sometimes associated with lichen planus-like lesions on the skin, nails, and mucous membranes (see Chapter 15: InflAMmatory Eruptions of Unknown Cause).

Pathogenesis

Like lichen planus, LPP is thought to result from a cell-mediated immune response to an unknown trigger.

Clinical Manifestations

Patients with LPP initially complain of erythema and burning that is then followed by the development of patchy alopecia, most commonly on the vertex of the scalp.
Typically, there is perifollicular erythema and scale (Fig. 19.16).
Tufting or polytrichia, which are clumps of hairs caused by surrounding scarring, is often seen.
There is great variability in the severity of LPP.
LPP tends to be progressive but tends to “burn out” after several years.
Frontal fibrosing alopecia, is a clinical variant of LPP which affects the frontal area of the scalp and is most often seen in elderly Caucasian women (Fig. 19.17). Frontal fibrosing alopecia is not associated with on the skin.

Diagnosis

A scalp biopsy is often necessary to make the diagnosis.
Serologic studies to help exclude connective tissue disease are recommended.

Differential Diagnosis

Other causes of scarring alopecias: central centrifugal cicatricial alopecia (see below), tinea capitis, cutaneous sarcoidosis, and traction alopecia.

Management

Superpotent topical or intralesional corticosteroids are the first line of therapy for LPP.
Doxycycline or minocycline (100 to 200 mg/day) is often effective in mild cases of LPP.
Other therapies that have been used include cyclosporine, methotrexate, hydroxychloroquine, dapsone, pioglitazone (a PPAR- agonist), and oral isotretinoin.

Central Centrifugal Cicatricial Alopecia

Basics

Central centrifugal cicatricial alopecia (CCCA) is the current term used to describe a type of scarring alopecia that is mostly seen in African-American women believed to be triggered by caustic hair treatment chemicals and/or heat from hot combs in a susceptible person. Previously, the traditional terms hot-comb alopecia and follicular degeneration syndrome were coined to describe this condition.

Pathogenesis

It is now known that patients who develop CCCA have hair follicles that show premature desquamation of the inner root sheath even before clinical symptoms are seen.
This abnormality predisposes the affected follicles to injury or inflammation in response to chemicals found in commercial styling products and relaxers or excessive heat from hot combs.

Clinical Manifestations

As the name implies, CCCA presents with patches of scarring alopecia on the central vertex of the scalp, and progresses centrifugally outward (Fig. 19.18).
Early hair loss is usually asymptomatic and gradual. Most patients report only mild, occasional pruritus or pain.
Pustules, itching, and crusting may occur in rapidly progressive disease.
Hypopigmentation and hyperpigmentation may be seen in affected scarred scalp areas.
Tufting or polytrichia within the alopecic area can also be noted.

Diagnosis

Diagnosis is based on the following:

Clinical appearance.
History of hair reshaping techniques.
Scalp biopsy, if necessary.

Differential Diagnosis

Other causes of scarring alopecias: chronic cutaneous lupus erythematosus (see above), lichen planopilaris (see above), tinea capitis, cutaneous sarcoidosis, and traction alopecia.

Management

Management of CCCA consists of removing, changing, or eliminating the damaging hairstyle practices as well as reducing the inflammation. Encouraging the patient to change hairstyle practices requires sensitivity as well as a good understanding of the different hairstyles and practices of black women.
Ideally, natural hairstyles that do not place traction on the hair shaft are recommended.
Use of mild, lye-free chemical relaxants once every 2 months is suggested, and the use of heat-relaxing devices such as hot combs and hood dryers should be discouraged.
Anti-inflammatory therapy with minocycline or doxycycline, as well as topical or intralesional corticosteroids are helpful in the initial phases.
Once the inflammation is completely resolved, hair transplantation into scarred areas can be used to improve cosmesis.

Helpful Hints

Although a great majority of black women are using or have used chemical and thermal relaxers, a diagnosis of CCCA should not be made presumptively. A scalp biopsy for H/E staining and direct immunofluorescence, a negative PAS stain, as well as negative or nonreactive connective tissue serologies, helps to rule out other causes of scarring alopecias.
The importance of early diagnosis and prompt initiation of treatment for a scarring alopecia is of the utmost importance. Once the follicle is replaced by scar tissue, the hair loss is irreversible.

Traction Alopecia

Basics

Traction alopecia is seen almost exclusively in African-American and African-Caribbean women of all ages, who are more likely to braid their hair.
The condition results from the prolonged and repeated trauma to the hair follicle by hairstyles such as cornrows, braiding, and tight ponytails.
The persistent physical stress of traction injury caused by tight rollers, tight braiding, or ponytails causes hair loss.

Clinical Manifestations

Traction alopecia is manifested by a symmetric pattern of hair loss, with broken hairs.
A characteristic border of residual hairs is often at the distal margin of the hair loss (Figs. 19.19A,B).
Traction pattern: alopecia is evident at the temples and along the frontal hairline. Hair loss later extends to the vertex and occipital areas.
A combination of these patterns may be seen if both traction and hot combs or chemicals are used.

Diagnosis

The clinical presentation and history are usually sufficient to make the diagnosis.

Differential Diagnosis

Chemical or Heat Induced Traumatic Alopecia

History of chemical or heat applications.
Hair loss is more irregular (less symmetric) and reflects the areas where the chemicals or hot comb were applied.

Management

Early intervention and discontinuation of the damaging hairstyles are the mainstays of therapy, because delay may result in irreversible hair loss.

Helpful Hint

A rim of residual hair is often present. They are short hairs that cannot be “grabbed” by rollers.

Cutaneous Sarcoidosis (see Chapter 34: Cutaneous Manifestations of Systemic Disease)

Basics

Hair loss due to cutaneous sarcoidosis may closely resemble that of other scarring alopecias discussed in this chapter.
Sarcoidosis of the scalp is most commonly noted in African-American women and in patients who have cutaneous or noncutaneous involvement elsewhere, such as pulmonary sarcoidosis.

Clinical Manifestations

Scalp lesions may present with a variety of morphologies, including alopecic papules, nodules, plaques, and infiltrated scars.

Diagnosis

A scalp biopsy demonstrating noncaseating granulomas may be necessary to distinguish sarcoidosis from other causes of scarring alopecia.
Evidence of cutaneous sarcoidosis elsewhere on the body or systemic involvement can support the diagnosis.
Serologic studies to exclude connective tissue diseases are recommended.

Management (see Chapter 34: Cutaneous Manifestations of Systemic Disease for Other Treatment Modalities)

Potent topical, intralesional, or oral corticosteroids may be used to control sarcoidosis of the scalp.

Folliculitis Decalvans

Basics

Folliculitis decalvans presents as a scarring patch of alopecia surrounded by follicular pustules.
This condition does not merely represent a bacterial folliculitis; rather, an abnormal immune response to possible staphylococcal antigens may be involved.

Clinical Manifestations

Successive crops of peripheral pustules result in an expanding patch or patches of scarring alopecia (Fig. 19.20).

Diagnosis

Bacterial cultures from the follicular pustules often grow Staphylococcus aureus.
A scalp biopsy for H/E staining demonstrates a scarring alopecia with a neutrophilic infiltrate.

Differential Diagnosis

Although rare in adults, it is important to rule out tinea capitis, which can also present as a pustular scarring alopecia.

Management

Prolonged antistaphylococcal treatment with monotherapy (dicloxacillin, minocycline, cephalexin) or combination therapy (rifampin plus clindamycin or cephalexin).
Topical antibiotic agents, and topical and intralesional corticosteroids may be used in conjunction with systemic therapy.

Outline
Chronic Cutaneous Lupus Erythematosus (see also Chapter 34: Cutaneous Manifestations of Systemic Disease)
Lichen Planopilaris
Central Centrifugal Cicatricial Alopecia
Traction Alopecia
Cutaneous Sarcoidosis (see Chapter 34: Cutaneous Manifestations of Systemic Disease)
Folliculitis Decalvans

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Basics ⬇

Pathogenesis ⬆ ⬇

Clinical Manifestations ⬆ ⬇

Other Information ⬆