Primary Cicatricial Alopecia: Clinical Features and Management

Elizabeth K. Ross, MD

Disclosures

Dermatology Nursing. 2007;19(2):137-143. 

In This Article

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.

Lichen planopilaris. Source: Photo courtesy of Paradi Mirmirani, MD.

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.

Frontal fibrosing alopecia.

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.

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 high-potency 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).

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