Cicatricial Alopecia: An Approach to Diagnosis and Management

By Vera Price

This is the first and only book on the diagnosis and treatment of cicatricial alopecia, written by leading experts in the field. This highly illustrated and practical text helps residents and practicing dermatologists to accurately diagnose and treat the scarring alopecias.

• Language: English
• Publisher: Springer
• Release date: Mar 24, 2011
• ISBN: 9781441983992

Book preview

Vera Price and Paradi Mirmirani (eds.)Cicatricial AlopeciaAn Approach to Diagnosis and Management10.1007/978-1-4419-8399-2_1© Springer Science+Business Media, LLC 2011

1. Introduction

Vera Price¹   and Paradi Mirmirani¹, ², ³  

(1)

Department of Dermatology, University of California, San Francisco, San Francisco, California 94115, USA

(2)

Department of Dermatology, Case Western Reserve University, Cleveland, OH, USA

(3)

Department of Dermatology, The Permanente Medical Group, 975 Sereno Drive, Vallejo, California 94589, USA

Vera PriceProfessor (Corresponding author)

Email: [e-mail]

Paradi MirmiraniStaff Physician

Email: [e-mail]

Abstract

Cicatricial alopecias (scarring alopecias) encompass a diverse group of inflammatory disorders that cause permanent destruction of the pilosebaceous unit and irreversible hair loss. They may be primary or secondary. In the primary group, the hair follicle is the target of a folliculocentric inflammatory attack that results in replacement of the follicle with fibrous tissue. The secondary scarring alopecias are the result of a non-folliculocentric process or external injury; follicular destruction is secondary and incidental, as in severe infections (kerion), infiltrations (tumors, metastatic cancer, sarcoid), physical injuries (thermal burns, radiation, traction). In this monograph, the focus is on the primary scarring alopecias.

How Are the Cicatricial Alopecias Classified?

Cicatricial alopecias (scarring alopecias) encompass a diverse group of inflammatory disorders that cause permanent destruction of the pilosebaceous unit and irreversible hair loss. They may be primary or secondary. In the primary group, the hair follicle is the target of a folliculocentric inflammatory attack that results in replacement of the follicle with fibrous tissue. The secondary scarring alopecias are the result of a non-folliculocentric process or external injury; follicular destruction is secondary and incidental, as in severe infections (kerion), infiltrations (tumors, metastatic cancer, sarcoid), physical injuries (thermal burns, radiation, traction). In this monograph, the focus is on the primary scarring alopecias.

A first step in improving dialogue among clinicians and investigators was the workshop on cicatricial alopecia sponsored by the North American Hair Research Society in 2001. This workshop proposed a working classification of the cicatricial alopecias based on the predominant cellular infiltrate, whether lymphocytic, neutrophilic, mixed, or absent in the end stage. After this workshop, the term cicatricial alopecia was adopted in the US, but the terms cicatricial and scarring are interchangeable. This classification continues to evolve, and a modified version is followed here (Table 1.1). The classification is currently used as a guide for selecting treatment.

Table 1.1

Working classification of primary cicatricial alopeciaa

a The above is a modification of the working classification of primary cicatricial alopecia which was proposed by the North American Hair Research Society in 2001. This modified classification is currently used by us as a guide for selecting treatment

b Not a primary cicatricial alopecia

What Are the Demographics of Patients with Cicatricial Alopecia?

The incidence of the cicatricial alopecias is not precisely known. The annual incidence rate of lichen planopilars (LPP) was reported in four tertiary hair research centers in the United States (Table 1.2). Annual incidence rate of LPP was defined as the percentage of new patients with biopsy-proven LPP and clinicopathologic correlation among all new patients with hair loss seen over a 1-year period. In these four centers, the annual incidence of LPP varied from 1.15 to 7.59%, which attests to the relative rarity of LPP.

Table 1.2

Annual incidence of LPP in four tertiary hair research centers

Reprinted from Ochoa BE, et al. Lichen planopilaris: annual incidence in four hair referral centers in the United States. J Am Acad Dermatol. 2008;58(2):353, copyright 2008, with permission from Elsevier

LPP Lichen planopilaris

a Includes cases of LPP, frontal fibrosing alopecia, and pseudopelade (Brocq)

b Number rounded up from 2.6

In a survey of 159 patients with cicatricial alopecia seen in the Dermatology Department, University of California, San Francisco between 2003 and 2005, the relative incidence of the ­various cicatricial alopecias, gender predisposition, racial incidence, and age of onset are shown in Figs. 1.1–1.4. However, the relative incidence of the various scarring alopecias shown in Fig. 1.1 represents patients who were referred to and seen in one geographic site (the San Francisco Bay area). This incidence will vary widely at different geographic sites, depending on ethnic populations and referral patterns. The incidence of CCCA, for example, will increase with a higher population of African ancestry. At the same time, gender predisposition, racial incidence, and the age of onset are probably generally representative in Figs. 1.2–1.4.

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Fig. 1.1

Survey of 159 patients with cicatricial alopecia: Relative incidence of various cicatricial alopecias seen in one geographic site, the San Francisco Bay area, between 2003 and 2005. This incidence will vary at different geographic locations, depending on ethnic populations and referral patterns

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Fig. 1.2

Survey of 159 patients with cicatricial alopecia: gender predisposition

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Fig. 1.3

Survey of 159 patients with cicatricial alopecia: ethnicity

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Fig. 1.4

Survey of 159 patients with cicatricial alopecia: age of onset

Why Is Hair Loss in Cicatricial Alopecia Irreversible?

As with non-scarring alopecias such as androgenetic alopecia and alopecia areata, the cicatricial alopecias occur in healthy people and are not contagious. However, unlike androgenetic alopecia and alopecia areata, the hair loss is irreversible. Why is this?

The location of the perifollicular inflammatory infiltrate determines the irreversibility and reversibility of hair loss (Fig. 1.5). Destruction of the hair follicle stem cells and the sebaceous gland leads to permanent hair loss. For this reason, early and aggressive treatment of the scarring alopecias is key and is aimed at controlling the inflammatory infiltrate to minimize the extent of permanent hair loss.

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Fig. 1.5

In primary cicatricial alopecia, the inflammatory infiltrate is located around the upper part of the hair follicle around the infundibulum and isthmus and results in destruction of the hair follicle stem cells in the hair bulge and the sebaceous gland. Destruction of these structures leads to permanent hair loss. In contrast, alopecia areata is always potentially reversible because the inflammatory infiltrate is located around the hair bulb, and the sebaceous gland and the hair follicle stem cells are not affected. Courtesy of Jeff Donovan, MD

Takeaway Pearls

The terms cicatricial and scarring are interchangeable.

Hair loss in cicatricial alopecia is irreversible because the inflammatory infiltrate is located around the infundibulum and isthmus and results in destruction of the hair follicle stem cells and the sebaceous gland.

Alopecia areata is always potentially reversible because the inflammatory infiltrate is located around the hair bulb, and the hair follicle stem cells and sebaceous gland are not affected.

Suggested Reading

Ochoa BE, King LE Jr, Price VH. Lichen planopilaris: annual incidence in four hair research centers in the United States. J Am Acad Dermatol. 2004;50:25–32.CrossRef

Olsen EA, Bergfeld WF, Cotsarelis G, et al. Summary of North American Hair Research Society (NAHRS)-sponsored Workshop on Cicatricial Alopecia, Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol. 2003;48:103–10.PubMedCrossRef

Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg. 2006;25:41–50.PubMedCrossRef

Vera Price and Paradi Mirmirani (eds.)Cicatricial AlopeciaAn Approach to Diagnosis and Management10.1007/978-1-4419-8399-2_2© Springer Science+Business Media, LLC 2011

2. Clinical Assessment of the Patient

Vera Price¹   and Paradi Mirmirani¹, ², ³  

(1)

Department of Dermatology, University of California, San Francisco, San Francisco, California 94115, USA

(2)

Department of Dermatology, Case Western Reserve University, Cleveland, OH, USA

(3)

Department of Dermatology, The Permanente Medical Group, 975 Sereno Drive, Vallejo, California 94589, USA

Vera PriceProfessor (Corresponding author)

Email: [e-mail]

Paradi MirmiraniStaff Physician

Email: [e-mail]

Abstract

The patient with hair loss invariably complains I am losing my hair, which can mean vastly different things in different patients. Every patient with hair loss should have the following baseline lab studies: CBC, TSH, ferritin, and vitamin D 25OH because you do not want to miss other possible contributing factors of hair loss.

Keywords

OstiaScalp biopsyPatient evaluationHair CardAnagen hairsTug Test

The patient with hair loss invariably complains I am losing my hair, which can mean vastly different things in different patients. Every patient with hair loss should have the following baseline lab studies: CBC, TSH, ferritin, and vitamin D 25OH because you do not want to miss other possible contributing factors of hair loss.

Listen to the patient carefully. A common feedback from patients with cicatricial alopecia is that they have seen many doctors who listened only half heartedly and then sent them on their way. Taylor the consultation to uncover the chronology and specifics of each patient. It will vary depending on the problem and the patient.

The clinical examination begins as soon as you enter the room. Note the patient’s hair style. Position all patients with hair problems in a chair, not on the exam table, in order to see the hair and scalp from above (unless you are a giraffe!). Good lighting is essential; ideally have a magnifying light and a dermatoscope. Before taking the full history, it often helps to look at the patient’s scalp briefly to establish whether follicular ostia are present or absent. Diminished or absent ostia are the hallmark of cicatricial alopecia, and this helps to guide the history taking. If a cicatricial alopecia is suspected, a scalp biopsy is the essential first step in assessing the patient.

If Time Is Limited On the Initial Visit, Take the Scalp Biopsy On the First Visit, and Complete the Evaluation at Next Visit

Scalp Biopsy

Select an active hair-bearing area with positive anagen pull test (preferred but not essential); avoid old, bare, late-stage areas. Infiltrate biopsy site with 1% lidocaine with epinephrine, and always wait 10 min for maximum vasoconstriction. Position the patients sitting down, leaning over the examination table, and bracing their head with their hands (like The Thinker) (Fig. 2.1). If an assistant is available, have her hold gauze squares and Q-tip to help with hemostasis. Sample down to subcutis. Take one deep 4-mm punch biopsy in the direction of hair growth for horizontal sectioning and H & E staining, or two 4-mm punch biopsies for both horizontal and vertical sectioning (depending on the preference of your dermatopathologist). Close with 3-0 blue suture (helps in finding the site).

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Fig. 2.1

Positioning for scalp biopsies and scalp injections. Have patients sit on a stool or a chair and lean over the