Primary Cicatricial Alopecia: Clinical Features and Management

Elizabeth K. Ross, MD

Disclosures

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

In This Article

Abstract and Introduction

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.

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 be tween 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.

Close-up of lichen planopilaris showing perifollicular scale and erythema around affected follicles, and scarring marked by absent follicular ostia in areas without hair. Source: Photo courtesy of Paradi Mirmirani, MD.

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