Firm, indurated scalp lesion with hair loss

Lichen planopilaris (LPP), also called follicular lichen planus or lichen follicularis, is a subtype of lichen planus. The condition, first described in 1895, is a cutaneous disorder selectively affecting the follicles of the scalp, causing a scarring and irreversible alopecia....

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Lichen planopilaris (LPP), also called follicular lichen planus or lichen follicularis, is a subtype of lichen planus. The condition, first described in 1895, is a cutaneous disorder selectively affecting the follicles of the scalp, causing a scarring and irreversible alopecia. Although LPP is a rare condition overall, it is one of the most common causes of cicatricial alopecia.

The three subtypes of LPP are classic LPP, frontal fibrosing alopecia, and Graham-Little-Piccardi-Lasseur syndrome (GLPLS). Classic LPP presents with perifollicular erythema and progressive scarring, often on the central scalp. Frontal fibrosing alopecia is seen most commonly in older women and features a bandlike pattern of alopecia in the frontal and temporal regions only. GLPLS includes cicatricial alopecia of the scalp, noncicatricial alopecia of the axillary and pubic hair, and keratosis pilaris of the skin of the trunk and extremities.

Women tend to be afflicted with LPP more often than do men. Median age of onset is 40 to 60 years. Physical exam findings include perifollicular erythema and scaling of the scalp with acuminate keratotic plugs. As the inflammation resolves, it is replaced with a smooth, white, atrophic patch with a complete loss of hair follicles within. A perifollicular violaceous erythema is present at the periphery of these lesions, indicating continued progression of the disease. Disease activity may be centralized or multifocal with smaller scattered patches that eventually coalesce into a larger area of alopecia.

Dermoscopy is a useful tool for clinically observing the lack of follicular orifices and the perifollicular erythema. In addition, false peripilar cysts may be observed on the hair shaft.¹

Most individuals present in great distress over sudden hair loss. Scalp itching, burning, tenderness, or discomfort may also be reported. It is estimated that 7% to 27% of patients with LP have evidence of oral involvement with lacy white lesions on the buccal mucosa, while 20% to 40% have evidence of cutaneous involvement with the classic polygonal flat-topped papules.²

The pathophysiology of LPP is unclear, but an autoimmune etiology is thought to play a role. Rarely, LPP may be triggered by a pharmacologic agent.¹

Histopathologic examination is often necessary to make an accurate diagnosis. Biopsy of active disease tissue will show an inflammatory cell infiltrate surrounding the infundibula of the hair follicles. Cytoid bodies may fill the remnants of the fibrous tract. Direct immunofluorescence (DIF) testing is often negative, or rarely it may reveal shaggy linear fibrin and cytoid bodies at the dermo-epidermal junction. To identify active disease, the biopsy must be performed on the perimeter of the affected area. Biopsy specimens from the center atrophic area of alopecia will be nondiagnostic and reveal scar tissue. A 4-mm punch should be the minimum-size biopsy performed.

Differential diagnoses include seborrheic dermatitis and alopecia areata at the very initial stages of the disease when the scarring is not yet obvious. In later stages of the disease, LPP must be differentiated from discoid lupus erythematosus, central centrifugal cicatricial alopecia, and folliculitis decalvans. Biopsy and DIF testing will help distinguish these conditions from LPP.

Treatment of LPP can be challenging, particularly since the disease tends to be slowly progressive and relapses are common. Although there are no known cures or treatments to reverse the hair loss associated with LPP, treatments are beneficial in slowing disease progress. Such high-potency topical steroids as clobetasol applied twice a day and then tapered down, or intralesional corticosteroid injections administered monthly, are the mainstays of treatment. Recently, the role of oral tetracycline has been reviewed.³ Oral retinoids have also been shown to be effective. End-stage treatment of inactive disease may include hair transplants and scalp reduction.

The clinician must be aware of the potentially severe psychological distress caused by LPP. Referral to a local or national alopecia organization can help patients contact others suffering from the same disease. In addition, patients may benefit from referral to a medical wig specialist.

Two 4-mm punch biopsies confirmed the diagnosis of LPP in the woman in this case. She was started on twice-daily clobetasol topical solution for four weeks and then tapered down to daily clobetasol for another four weeks. Her scalp was noted to have a dramatic decrease in peripheral erythema, and the patch of alopecia was stable in size. The long-term treatment goal was to slowly and continuously decrease the topical steroid solution while preventing a relapse in disease activity.

Esther Stern, NP, is a family nurse practitioner at Advanced Dermatology & Skin Surgery, P.C., in Lakewood, N.J.