Continuing Medical EducationUpdate on primary cicatricial alopecias
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,
References (403)
- et al.
Scarring alopecia
Dermatol Clin
(1987) Cicatricial alopecia
Dermatol Clin
(1996)- et al.
Summary of North American Hair Research Society (NAHRS)-sponsored workshop in cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001
J Am Acad Dermatol
(2003) Cicatricial alopecia: clinico-pathological findings and treatment
Clin Dermatol
(2001)- et al.
Primary cicatricial alopecias: clinicopathology of 112 cases
J Am Acad Dermatol
(2004) - et al.
Hair follicle stem cells
J Invest Dermatol
(2003) - et al.
Human hair follicle bulge cells are biochemically distinct and possess an epithelial stem cell phenotype
J Investig Dermatol Symp Proc
(1999) - et al.
Upper human hair follicle contains a subpopulation of keratinocytes with superior in vitro proliferative potential
J Invest Dermatol
(1993) - et al.
Location of stem cells of human hair follicles by clonal analysis
Cell
(1994) - et al.
Involvement of follicular stem cells in forming not only the follicle but also the epidermis
Cell
(2000)
Asebia-2J (Scd1ab2J): a new allele and a model for scarring alopecia
Am J Pathol
Defolliculated (dfl): a dominant mouse mutation leading to poor sebaceous gland differentiation and total elimination of pelage follicles
J Invest Dermatol
Genetic disorders of keratin: are scarring alopecias a sub-set?
J Dermatol Sci
Vertical and transverse sections of alopecia biopsy specimens: combining the two to maximize diagnostic yield
J Am Acad Dermatol
Medical pearl: fluorescence microscopy of hematoxylin-eosin-stained sections
J Am Acad Dermatol
Skin manifestations of SLE
Clin Rheum Dis
Keratotic lupus erythematosus: treatment with isotretinoin
J Am Acad Dermatol
Systemic lupus erythematosus in patients with chronic cutaneous (discoid) lupus erythematosus. Clinical and laboratory findings in seventeen patients
J Am Acad Dermatol
Papulonodular dermal mucinosis in lupus erythematosus
J Am Acad Dermatol
Lupus band test: anatomic regional variations in discoid lupus erythematosus
J Am Acad Dermatol
Patients with cutaneous lupus erythematosus who smoke are less responsive to antimalarial treatment
J Am Acad Dermatol
Isotretinoin for refractory lupus erythematosus
J Am Acad Dermatol
Treatment of resistant discoid lupus erythematosus of the palms and soles with mycophenolate mofetil
J Am Acad Dermatol
Uses of vitamins A, C, and E and related compounds in dermatology: a review
J Am Acad Dermatol
Low-dose intralesional interferon alfa for discoid lupus erythematosus
J Am Acad Dermatol
Treatment of severe cutaneous lupus erythematosus with a chimeric CD4 monoclonal antibody, cM-T412
J Am Acad Dermatol
Treatment of localized discoid lupus erythematosus with tazarotene
J Am Acad Dermatol
The effect of hair loss on quality of life
Eur Acad Dermatol Venereol
Cicatricial (scarring) alopecias
Cicatricial alopecia
Scarring alopecia: a classification based on microscopic criteria
J Cutan Pathol
A new look at scarring alopecia
Arch Dermatol
Controls of hair follicle cycling
Physiol Rev
Distribution of Langerhans cells in human hair follicle
J Cutan Pathol
Hereditary absence of sebaceous glands in the mouse
Science
Asebia-J is known to be very close to ru on chromosome 19
Mouse News Letter
The asebia (ab, abJ) mutations, chromosome 19
Scd1 is expressed in sebaceous glands and is disrupted in the asebia mouse
Nat Genet
The bareskin (Bsk) mutation, chromosome 11
Bareskin (Bsk)
Mouse News Letter
Hair follicle companion layer: reacquainting an old friend
J Invest Dermatol
Increased expression of keratin 16 causes anomalies in cytoarchitecture and keratinization in transgenic mouse skin
J Cell Biol
Skin and hair follicle integrity is crucially dependent on beta 1 integrin expression on keratinocytes
Embo J
Insights from the asebia mouse: a molecular sebaceous gland defect leading to cicatricial alopecia
J Cutan Pathol
Hair follicle biology, the sebaceous gland, and scarring alopecias
Arch Dermatol
Transverse microscopic anatomy of the human scalp
Arch Dermatol
Evaluating and describing transverse (horizontal) sections
Scarring alopecia and the dermatopathologist
J Cutan Pathol
Subtle clues to diagnosis by immunopathology: scarring alopecia
Am J Dermatopathol
Differential patterns of elastic fibers in scarring and non-scarring alopecias
J Cutan Pathol
Cited by (217)
Hidradenitis suppurativa and follicular occlusion syndrome: Where is the pathogenetic link?
2023, Clinics in DermatologyDiagnosis and treatment of scarring alopecia
2023, Procedures in Cosmetic Dermatology: Hair RestorationHistopathologic and dermoscopic features of 42 cases of folliculitis decalvans: A case series
2021, Journal of the American Academy of DermatologyCitation 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.
Scarring Alopecia: Diagnosis and New Treatment Options
2021, Dermatologic ClinicsDiagnostic delay, comorbid hidradenitis suppurativa and the prognostic value of bacterial culture in folliculitis decalvans: A cohort study
2023, JDDG - Journal of the German Society of DermatologyPrevalence and incidence of comorbid diseases and mortality risk associated with lichen planopilaris: A Korean nationwide population-based study
2023, Clinical and Experimental Dermatology
Supported by the Canadian Hair Research Foundation.
Disclosure: Dr Shapiro is a consultant for Pfizer, Inc.