Primary Cicatricial Alopecia: Clinical Features and Management

The primary cicatricial alopecias are an uncommon, complex group of disorders that result in permanent destruction of the hair follicle, usually involving scalp hair alone.

Prompt diagnosis and treatment are needed to help thwart continued hair loss and the distress that often accompanies this hair loss.

Nurses can facilitate the diagnostic and treatment process and, through educational and emotionally supportive measures, have a meaningful, positive impact on the patient's well being.

Objectives

This continuing nursing educational (CNE) activity is designed for nurses and other health care providers who care for and educate patients and their families about primary cicatricial alopecia. For those wishing to obtain CNE credit, an evaluation follows. After studying the information presented in this article, the nurse will be able to:

1. Identify the clinical hallmark of scarring alopecia.

2. Define the difference between primary and secondary cicatricial alopecia.

3. State the essential diagnostic test done when cicatricial alopecia is suspected clinically.

4. Identify the nurse's role in management of cicatricial alopecia.

Hair is a defining feature of individual character. It can reveal aspects of self, health, ethnicity, and socioeconomic status. It is not surprising then, that self-esteem and confidence can suffer in those who experience hair loss. Patients who seek medical attention for their problem arrive at their clinic visit eager to learn the diagnosis, and are anxious about how much more hair loss they can expect and whether effective treatment options are available. In the 10 to 20-minute slot that is commonly allotted for general dermatology patient visits, delivery of effective and compassionate care is a challenge. Nurses are essential to making the visit complete, by first obtaining informed histories so that the physician can then hone in on the specifics, and then by following the visit with educational and emotional support. Knowledge about the varied types of hair loss and treatment options is requisite to this effort.

Hair loss is generally divided into two groups: (a) non-scarring hair loss, such as alopecia areata and the familiar inherited male and female pattern hair loss (androgenetic alopecia), and (b) scarring or cicatricial hair loss. Unlike non-scarring alopecia, cicatricial alopecia results in permanent destruction of the hair follicle. Causes of cicatricial alopecia are considered either primary or secondary. In primary cicatricial alopecia, the hair follicle is the target of inflammatory destruction, with little effect of the disease process on other components of the dermis. In secondary cicatricial alopecia, the hair follicle is an "innocent bystander" and is destroyed indirectly. Examples of secondary cicatricial alopecia include burns and blistering disorders such as pemphigus vulgaris. In all cases of suspected cicatricial alopecia, a skin biopsy should be done to confirm the scarring nature of the condition and to help guide treatment. The focus of this article is on the primary cicatricial alopecias.

The primary cicatricial alopecias are a complex group of disorders with little known about the cause, pathogenesis, and best treatment options. The reason for this lack of knowledge is that most of these conditions are encountered less frequently in the clinic than other types of hair loss. Much of what is known has been culled from a few large hair specialty clinics' experience and small case reports (Bergfeld & Elston, 2003; Ross, Tan, & Shapiro, 2005). A familiarity with the clinical features of these disorders is essential to prompt recognition and the timely institution of appropiate treatment to thus minimize scarring to the greatest extent possible.

In an effort to increase understanding about the primary cicatricial alopecias, a workshop sponsored by the North American Hair Research Society was held in 2001 and resulted in the development of a working classification system to standardize communication between researchers and clinicians on the subject. Each entity is classified according to the predominant inflammatory cell involved (lymphocytic, neutrophilic, mixed) (Olsen et al., 2003) (see Table 1). Since then, a number of clinical and basic research articles have been published on novel aspects of certain diseases, and new theories have been advanced on the pathogenesis of these diseases and primary cicatricial alopecia in general (Ross et al., 2005). In 2005, researchers and clinicians convened for the first time at the National Institutes of Health to review these conditions and to recommend directions for future research (Stenn, Cotsarelis, & Price, 2006). This exciting colloquium was spearheaded by the Cicatricial Alopecia Research Foundation (C.A.R.F.).

In this article, clinical aspects of six of the more common types of primary cicatricial alopecia are discussed: lichen planopilaris, pseudopelade of Brocq, central centrifugal cicatricial alopecia, discoid lupus erythematosus, folliculitis decalvans, and acne keloidalis. These diseases can usually be distinguished clinically, and all have in common the loss of follicular orifices or ostia in areas of involvement (see Figure 1). However, histologically, a recent study suggests that differentiation of individual entities beyond separating those with predominantly lymphocytic or neutrophilic inflammation may not be possible (Mirmirani et al., 2005). Ultimately, they all show scarred destruction of follicles.

Lichen Planopilaris

Lichen planopilaris is a disorder of largely unknown cause, of which there are three main subtypes: lichen planopilaris, frontal fibrosing alopecia, and Graham-Little syndrome.

In lichen planopilaris, the onset is usually in adult women between ages 30 and 60 years. The scalp is often the only site involved, although the skin elsewhere, mouth, and genitalia may be affected. Pruritus, burning, and pain are often severe. Disease can be multifocal or diffuse, often starting on the central scalp. Hair follicles are tightly cuffed by white scale (called perifollicular hyperkeratosis) and erythema; hair loss may or may not be evident at this stage (see Figure 1). Affected hairs can often be easily extracted with minimal traction (this is called a pull test). The hair that is extracted may show a long glassy ensheathed root tip - the sign of a growing or anagen hair - that can be seen with the naked eye; this is a sign of active disease. In normal scalps, only a few resting or telogen hairs (which show a small white root tip) are yielded with this test. As the condition advances, hair loss becomes more evident, leaving white areas with absent follicular orifices; active disease is seen at the hair-bearing margin (see Figure 2). The course of the disease is generally slowly progressive and well camouflaged by the remaining hair. In other cases, a more rapid course may result in extensive loss within months.

Frontal fibrosing alopecia is a recently recognized form of primary cicatricial alopecia that is considered by some a type of lichen planopilaris based on clinical and histopathologic findings. This condition mostly affects postmenopausal women, but no connection to hormone status or the use of hormone replacement therapy has been found. Indeed, a few cases in premenopausal women and rare cases in men have been also reported.

Frontal fibrosing alopecia is marked by striking recession of the frontal and frontotemporal hairline. The affected skin shows loss of follicular ostia and is pale and shiny in contrast to chronically sun-exposed skin of the upper forehead (see Figure 3). The depth of the recession can range from a few to several centimeters. The hairs at the margin of this recession and those, usually few, remaining hairs in the receded area may show perifollicular erythema and/or scale - signs of active disease. Concomitant patchy or complete eyebrow loss is characteristic and observed frequently. Scarring hair loss in other scalp sites may also occur.

Graham-Little syndrome is classically considered a type of lichen planopilaris, but some have argued that it is a unique entity (Ross et al., 2005). This uncommon condition of adults is marked by a triad of patchy scarring hair loss of the scalp, nonscarring alopecia of the axillae and pubic area, and follicular papules with spinous scale on the trunk and extremities.

Treatment. As with all the primary cicatricial alopecias, treatment is based on the extent of the disease, activity, rate of progression, and age and general health of the patient. First-line treatment for localized, indolent disease is mid or highpotency topical corticosteroids. Intralesional injections of triamcinolone acetonide can be added or substituted if no benefit is derived after 4 weeks. In more rapidly advancing or extensive disease, an empiric trial of a systemic agent such as hydroxychloroquine, mycophenolate mofetil, cyclosporine, or isotretinoin may be tried. Oral prednisone can be used to slow and even stop activity in the short term, but cannot be used in the long term because of the chronic nature of this condition. Similar treatment approaches are used in frontal fibrosing alopecia and in Graham-Little syndrome (Ross et al., 2005). Treatment response is followed at each visit by tracking symptoms and signs, the pull test, and the extent of the disease process (Mirmirani, Willey, & Price, 2003).

Pseudopelade of B\rocq

This primary cicatricial alopecia is named after a French dermatologist who was the first to study the condition in great detail in the late 1800s. He and others appreciated similarities in its appearance to "pelade," the French term for alopecia areata, hence the name pseudopelade. The following description of the pseudopelade of Brocq will be based on Brocq et al.'s description of the condition.

Pseudopelade of Brocq is a scalp disorder of middle-aged adults. Unlike lichen planopilaris, patients with pseudopelade of Brocq rarely have symptoms and visible signs of inflammation. A positive pull test, which yields anagen hairs when the disease is active, is a useful sign, but is not always present. Presentations include small oval or large round patches; the affected scalp surface may resemble "footsteps in the snow" (see Figure 4) or the entire central scalp may be affected diffusely. The scarred areas are usually completely devoid of hair and the skin supple and white.

Treatment approaches are similar to those described for lichen planopilaris. Treatment response is judged by the extent of scalp involved as neither symptoms nor inflammatory signs of disease are usually available to judge efficacy. Sometimes a followup biopsy is used to assess treatment response.

Central Centrifugal Cicatricial Alopecia

This form of primary cicatricial alopecia is seen mainly in African-American women. Former names for the condition are hot-comb alopecia, follicular degeneration syndrome, and pseudopelade in African Americans. The cause is disputed, but some clinicians feel that chronic use of physically and chemically traumatic hair-care practices (for example, chemical straighteners) are involved in its development.

Like pseudopelade of Brocq, symptoms and outward signs of follicular inflammation are usually minimal. However, some patients complain of itch, tingling, or tenderness in the affected scalp. Occasionally, slight perifollicular hyperpigmentation may be seen. The central or vertex scalp is involved, usually starting in the midline. Gradual, symmetric, centrifugal spreading alopecia, in pseudopod-like extensions, occurs over many years. The affected scalp is shiny, flesh-colored, and soft to touch (see Figure 5).

High-potency topical corticosteroids may be tried first; intralesional injections of triamcinolone are added if the benefit is suboptimal. A combination of topical corticosteroid and an oral tetracycline has also been reported to be effective; the effect may take months to be appreciated. Although cessation of traumatic haircare practices may not impact the course of disease, the patient should be advised to adopt gentle hair care. If hair breakage is also present, the use of heat, chemicals, and traction should be minimized or avoided.

Discoid Lupus Erythematosus

Discoid lupus erythematosus (also called chronic cutaneous lupus erythematosus) predominantly affects adult women. Discoid lupus erythematosus on the scalp has the same appearance as discoid lupus elsewhere on the face and body. The scalp is the sole skin site affected in 11% to 20%. The extent can be focal or widespread. Patients typically complain of patchy hair loss associated with pruritus, burning, or stinging. Early disease is marked by a small bare patch that enlarges and shows erythema and follicular hyperkeratosis (called follicular plugging) in the center of the alopecic patch (see Figure 6). In active disease, the pull test usually yields anagen hairs. In late stages, the affected skin becomes atrophic, telangiectatic, and shows hypo and hyperpigmentaion - characteristic of discoid lupus. Recurrences are often seen in former areas of involvement. There is a 5% to 10% risk of developing systemic lupus erythematosus, which is more likely in those with widespread cutaneous disease.

Treatment of discoid lupus must be energetic and started quickly because the hair loss is potentially reversible (unlike hair loss in most other types of primary cicatricial alopecias). Localized active disease is generally managed with high-potency topical corticosteroids and/or intralesional injections of triamcinolone acetonide into affected hair-bearing scalp, usually with good effect in stopping the disease process and in regrowing the hair. Rapidly advancing or extensive discoid lupus requires the use of systemic agents. Hydroxychloroquine is usually tried first, as there are fewer side effects compared to other antimalarial drugs. Cigarette smoking can reduce efficacy, so patients should be encouraged to quit this habit. Treatment can take some time (4-8 weeks) to take effect, and may require "bridge" therapy with oral prednisone in the interim. The reader is referred to other sources for review of alternative treatment options, of which there are several (Callen, 2005; Ross et al., 2005).

Folliculitis Decalvans

Folliculitis decalvans is a scarring folliculitis of the scalp in which Staphylococcus aureus is commonly cultured. The onset is typically in young and middle-aged adults who are otherwise healthy and not prone to S. aureus infection elsewhere on the body.

Patients typically present with a complaint of "pimples" and hair loss of the scalp, which can be very painful and/or pruritic. The disease causes perifollicular erythema, pustules, crusting, and oozing.

Small areas of scarring alopecia gradually appear and may become extensive and impressive, leaving large areas of bare scalp with continued active disease at the periphery (see Figure 7). Tufted hair follicles, in which several hairs emerge from a single follicular opening, are seen in advanced disease, but this is a nonspecific feature seen in other scarring alopecias as well.

Management involves repeated culture of pustules to establish the identity and antibiotic sensitivity of the bacteria involved.

An appropriate antibiotic is instituted (for example, cephalexin) usually for a prolonged period, as relapse is common once treatment is stopped. Addition of topical mupirocin can help to improve outcome.

In those who fail this approach, a double antibiotic regimen with rifampin is used for 10 weeks (typically, with clindamycin, ciprofloxacin, or doxycycline; rifampin cannot be used alone because of rapid development of bacterial resistance). This regimen can produce a sustained disease-free period for months to years in some individuals. If nostril culture shows that the patient is a staph carrier, mupirocin ointment is applied in the nostrils twice daily for 5 days, and as indicated thereafter.

Acne Keloidalis

Acne keloidalis is most commonly seen in postpubertal males of African ancestry. The condition can resemble a scar or keloid, but is not considered a bone fide keloid.

In early disease, the patient usually complains of "pimples" affecting the nape of the neck, which can be pruritic and painful. Scattered pinpoint, soft to firm, brown or red papules or pustules with or without crusting are seen initially. The extent varies. In some individuals, large nodules and/or keloid-like plaques develop. Sinus tracts can occur within these larger lesions.

Treatment depends on the stage and extent of the disease. Early, mild papular or pustular disease can be managed with high-potency topical corticosteroids alone or combined with a topical antibiotic such as clindamycin. Larger, firm papules often require intralesional injection with triamcinolone acetonide. In patients with extensive cosmetically bothersome or symptomatic plaque disease, surgical excision is the only treatment option that is effective. The excision may be done in stages, depending on the size of the plaque, and tightness of the scalp if primary closure is done. Healing by secondary intention is another option.

Surgical Correction of Burnt-Out Cicatricial Alopecia

In patients who have cosmetically bothersome, burnt-out disease - which may be defined as asymptomatic, clinically inactive, stable hair loss for 2 years - hair transplantation and/or scalp reduction is a therapeutic option. The choice of the surgical approach is best done by the surgeon who will explain the pros and cons of the options to the patient.

Nurses' Role in Management

In addition to disease-specific treatments, several measures apply to management of all forms of cicatricial alopecia. First, in sun-exposed areas of scarring, patients should be instructed to apply a broad-spectrum sunblock (gel form) and to wear a tightly woven hat when outdoors. Second, treatment with topical minoxidil twice daily may be instituted by the physician to help thicken hairs in those with coexisting androgenetic alopecia, and to potentially prolong the growing phase in others; instructions on use should be reviewed to optimize benefit (Ross & Shapiro, 2005). Third, methods of cosmetic camouflage should be discussed to help ease the discomfort or embarrassment that patients feel who have lost their hair. These include (a) use of a partial or complete hairpiece, (b) cosmetic creams and fibers that match the hair color (c) highlighting darker hair with tones that more closely match scalp skin color so as to reduce the color contrast, and (d) alternative coiffures. Although a hairpiece is not always covered by insurance companies, a prescription from the physician for a "full scalp prosthesis" will usually avoid tax on the purchase. Significant restoration of self-esteem and confidence can result from all these measures. Provision of printed information on reputable local carriers of these products can be of tremendous help. Finally, patients should be referred to the Cicatricial Alopecia Research Foundation (C.A.R.F.) (www.carfintl.org), an excellent resource for individuals with primary cicatricial alopecia to learn more about their disease, research efforts, and upcoming patient-focused and scientific meetings on the subject. An informative medical pamphlet is also available from the foundation. An additional helpful resource is the North \American Hair Research Society (www.nahrsmembers.org).

Nurses are essential team members in the delivery of care to those with hair loss. This is particularly true in the case of the primary cicatricial alopecias, as these entities require prompt diagnosis and immediate treatment if the disease is active. Most patients are unaware of the existence of these entities when they arrive for their first clinic visit, and are shocked to learn from their physician that their hair loss is permanent in nature and, if clinically active, may worsen. It is important to explain at the outset that the aim of current treatments is to stop any symptoms and signs, and to arrest progression of hair loss. It is not possible to regrow the hair that is already lost. The good news is that these conditions do not affect general health, are usually limited to the scalp, and are not contagious.

Within the time constraints of a typical general dermatology visit, nurses can help both the physician and the patient in several important ways. Nurses can facilitate accurate diagnosis by obtaining careful and directed initial histories. They can also answer the myriad questions that arise after the clinic visit with the physician is over, which may include providing more information about the general nature of their condition, correct use of their prescribed medications, camouflage methods, sun-protection measures, and referral to support services. Patients leave the office feeling shored by both the physician and nurse's efforts to help them, and their ability to cope with their hair loss is made easier.

This article and the CNE answer/evaluation form are also available online at www.dermatologynursing.net

Note: The author reported no actual or potential conflict of interest in relation to this continuing nursing education article.

Table 1.

Proposed Working Classification of Primary Cicatricial Alopecia

Lymphocytic

Lichen planopilaris

Lichen planopilaris

Frontal fibrosing alopecia

Graham-Little syndrome

Classic pseudopelade (Brocq)

Central centrifugal cicatricial alopecia

Chronic cutaneous lupus erythematosus (discoid lupus erythematosus)

Alopecia mucinosa

Keratosis follicularis spinulosa decalvans

Neutrophilic

Folliculitis decalvans

Dissecting cellulitis/folliculitis (perifolliculitis capitis abscedens et suffodiens)

Mixed

Folliculitis (acne) keloidalis

Folliculitis (acne) necrotica

Erosive pustular dermatosis

Nonspecific (End Stage)

Note: Entities discussed are shown in italics.

Source: Adapted from Olsen et al., 2003.

References

Bergfeld, W.F., & Elston, D.M. (2003). Cicatricial alopecia (and other causes of permanent alopecia). In E.A. Olsen (Ed.), Disorders of hair growth: Diagnosis and treatment (2nd ed., pp. 368-398). New York: McGraw-Hill.

Callen, J.P. (2005). Management of "refractory" skin disease in patients with lupus erythematosus. Best Practice & Research. Clinical Rheumatology, 19(5), 767-784.

Mirmirani, P., Willey, A., Headington, J.T., Stenn, K.S., McCalmont, T.H., & Price, V.H. (2005). Primary cicatricial alopecia: Histopathologic findings do not distinguish clinical variants. Journal of the American Academy of Dermatology, 52(4), 637-643.

Mirmirani, P., Willey, A., & Price, V.H. (2003). Short course of cyclosporine in lichen planopilaris. Journal of the American Academy of Dermatology, 49(4), 667-671.

Olsen, E., Bergfeld, W., Cotsarelis, G., Price, V., Shapiro, J., Sinclair, R., et al. (2003). Summary of North American Hair Research Society (NAHRS)-sponsored workshop in cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001. Journal of the American Academy of Dermatology, 48(1), 103-110.

Ross, E.K., & Shapiro, J. (2005). Management of hair loss, Dermatology Clinics, 23(2), 227-243.

Ross, E.K., Tan, E., & Shapiro, J. (2005). Update on primary cicatricial alopecias. Journal of the American Academy of Dermatology, 53(1), 1-37.

Stenn, K.S., Cotsarelis G., & Price, V.H. (2006). Report from the cicatricial alopecia colloquium. The Journal of Investigative Dermatology, 126(3), 539-541.

Elizabeth K. Ross, MD, is a Private Dermatologist, Dermatology and Laser Center, NW, Bellingham, WA.