Continuing Medical Education
Update on primary cicatricial alopecias

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The cicatricial alopecias encompass a diverse group of disorders characterized by permanent destruction of the hair follicle and irreversible hair loss. Destruction of the hair follicle can result from primary, folliculocentric disease or as a secondary result. This article focuses on the former, or primary cicatricial alopecias. The cause and pathogenesis of many of these disorders are largely unknown. Although unique clinicopathologic features allow for accurate diagnosis in some cases, diagnostic certainty is often elusive and reflects the limits of present understanding. Classification of the primary cicatricial alopecias on the basis of pathology provides a diagnostic and investigational framework and, it is hoped, will facilitate future enlightenment. Details of classification, etiopathogenesis, clinicopathologic features, differential diagnosis, and practical management of the primary cicatricial alopecias will be discussed.

Learning objectives

Upon completion of this learning activity, participants should be familiar with the following aspects of the primary cicatricial alopecias: (1) the new, consensus-issued classification scheme, (2) current understanding about etiopathogenesis, (3) salient clinicopathologic features, (4) differential diagnosis, and (5) therapeutic management.

Section snippets

Classification of primary cicatricial alopecias

Several classification schemes for primary cicatricial alopecia exist in the literature. Distinction has been based on age of onset, clinical features, and pathology, among other phenomena.6, 7 In 2001, a group of leading hair clinicians, pathologists, and researchers, under the rubric of the North American Hair Research Society (NAHRS), issued a consensus opinion on classification of the primary cicatricial alopecias8 (Table I). Categorization is based on the principal inflammatory cell type

Lymphocytic cicatricial alopecias

Although we largely agree with the NAHRS consensus–recognized list of primary cicatricial alopecias, there are some minor points of departure. For one, as will be discussed in the relevant section to follow, we are not convinced that “central centrifugal cicatricial alopecia” (CCCA) is a distinct form of primary cicatricial alopecia; it may represent a common morphologic pattern seen in different diseases. In addition, categorization of keratosis follicularis spinulosa decalvans (KFSD) as a

Neutrophilic cicatricial alopecias

Folliculitis decalvans is a commonly encountered form of primary cicatrizing alopecia (10.7%-11.2%), unlike perifolliculitis capitis abscedens et suffodiens (1.4%-4.5%).9, 10

Mixed cicatricial alopecias

In this category, acne keloidalis, acne necrotica, and erosive pustular dermatosis of the scalp are discussed. We view classification of acne necrotica varioliformis as a mixed inflammatory primary cicatricial as somewhat controversial, given the infiltrate is predominantly lymphocytic. In addition, it is unclear whether erosive pustular dermatosis of the scalp results from primary, folliculocentric disease or from nonfollicular events such as exogenous trauma. Only acne keloidalis is seen with

Adjunctive treatment options

In addition to disease-specific treatment, a few adjunctive therapies may be of generic benefit. Suggested medical options have yet to be subjected to rigorously controlled studies.

In cases of primary cicatricial alopecia that coexist with AGA, a trial of topical minoxidil may improve cosmesis by enlarging miniaturized hairs.10 At the University of British Columbia hair clinic, topical 5% minoxidil solution (1 mL twice daily) is prescribed for 1 year and is continued in those with improvement.

Conclusion

The initial phase of many of the primary cicatricial alopecias is nonscarring and should be managed as a “trichologic emergency”2: prompt diagnosis and therapeutic intervention are key to thwarting permanent hair loss and a potential lifelong struggle with the psychosocial sequelae. Choice of treatment is dependent on diagnosis, age, severity of disease, and extent. In general, local treatment should be used in limited disease. Systemic modalities should be reserved for rapidly advancing,

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      Citation Excerpt :

      The present study of 42 patients with FD showed that men predominated among the younger patients, whereas women predominated among the elderly patients with a long disease duration, and the most frequently affected area was the vertex, in line with the findings of previous studies.2-4 Clinically, early lesions in FD are pinpoint pustules and crusts, and later patches of alopecia develop from the expansion of these areas, eventually resulting in central areas of scarring alopecia.9,10 In our study, most patients showed a single alopecic patch with central distribution, including early, ill-circumscribed, or confluent alopecic lesions and a larger alopecic patch showing intense, peripheral inflammation.

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    Supported by the Canadian Hair Research Foundation.

    Disclosure: Dr Shapiro is a consultant for Pfizer, Inc.

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