Background
A scarring alopecia is the final common pathway for many diseases (see list below). A scar is present associated with permanent hair loss. Histologically, there is a scar. Subtle clues may be present but frequently a diagnosis of scarring alopecia is all that can be rendered.
OUTLINE
PATHOGENESIS CHARACTERIZATION GENERAL
J Cutan Pathol. 2005 Nov;32(10):675-9. Abstract quote
Background: Lichen planopilaris (LPP) is the prototype of scarring alopecias that mainly target the infundibuloisthmic (bulge) region of hair follicle. Hair follicle stem cells have been shown to reside in the bulge.
Methods: We carried out this study to better define the possible pathogenetic role of the bulge in LPP. Thirty-five cases of LPP were studied. Multiple serial sections of biopsy specimens stained with hematoxylin and eosin, periodic acid Schiff-diastase, and Elastic van Gieson. The following immunostains were applied: CD3, CD4, CD8, CD1a, and Ki-67. Uninvolved follicles and normal scalp biopsy specimens served as normal controls.
Results: All cases showed a lichenoid lymphocytic infiltrate at the bulge region. The bulb area was spared. CD8(+) T cells were increased compared with CD4(+) T-cell population. Langerhans' cells were decreased. Proliferating stem cells, highlighted by Ki-67, showed a marked decrease in the bulge compared with uninvolved follicles.
Conclusion: Our study supports the finding that in LPP, the inflammatory infiltrate mainly involves the bulge region, where the stem cells reside. Once this area is damaged, the hair loses its potential of regrowth with resulting scarring alopecia. This is in contrast with inflammatory non-scarring alopecias such as alopecia areata, where the bulb region is targeted, sparing the stem cells.ANIMAL MODELS Insights from the asebia mouse: a molecular sebaceous gland defect leading to cicatricial alopecia
K. S. Stenn Juvenir Biosciences, Inc., Inc.,
Skillman, New Jersey, USA
J Cutan Pathol 2001;28 (9), 445-447 Abstract quote
The primary cicatricial alopecias have proven to be challenging for the clinician, dermatopathologist and the researcher – let alone the patient. If we are to improve our diagnostic and therapeutic tools for these very difficult disorders, we will need greater insight into their etiology.
Recent work with the mouse mutant, asebia, provides a model for cicatricial alopecia. In this model the pathology – perifollicular inflammation, sebaceous gland “destruction”, hair shaft granuloma, and cicatricial follicle drop-out – results from the mutation of one very important sebaceous gland gene. In the absence of this gene, the sebaceous gland is hypoplastic and normal sebum production is minimal to absent.
In this paper the relevance of this mutant to human alopecias is discussed and the point emphasized that the pathogenesis of some forms of human cicatricial alopecia could involve the sebaceous gland.
APOPTOSIS Massive lymphocyte-mediated apoptosis during the early stage of pseudopelade.
Pierard-Franchimont C, Pierard GE.
Dermatologica 1986;172(5):254-7 Abstract quote
The early evolving lesions of the hair follicles are described in pseudopelade, a type of cicatricial alopecia where clues for the diagnosis of lupus erythematosus or lichen plano-pilaris are lacking. A sudden and synchronized cell death of all the cells of the epithelial sheaths of the hair follicles occurs and is associated with a dense infiltration by lymphocytes. The epidermis remains uninvolved.
This unique aspect is most probably related to the mechanism of apoptosis. Its intensity and extension are much more pronounced than in lichen planus and in lupus erythematosus.
Pathogenesis of at least some cicatricial alopecias could therefore be related to massive apoptosis without evident and close relationship with the classical etiologies, i.e. lichen planus and lupus erythematosus.
HISTOPATHOLOGY CHARACTERIZATION GENERAL
J Am Acad Dermatol. 2005 Jul;53(1):1-37; quiz 38-40. Abstract quote
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.
- Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants.
Mirmirani P, Willey A, Headington JT, Stenn K, McCalmont TH, Price VH.
University of California, San Francisco, San Francisco, California, USA.
J Am Acad Dermatol. 2005 Apr;52(4):637-43. Abstract quote
BACKGROUND: Primary cicatricial alopecias encompass a group of disorders characterized by permanent destruction of the hair follicle. The varied clinical features and differences in terminology have led to difficulties in defining consistent clinicopathologic correlation.
OBJECTIVE: We sought clinicopathologic correlation of 6 clinically distinct primary cicatricial alopecias: lichen planopilaris, frontal fibrosing alopecia, pseudopelade (Brocq), central centrifugal alopecia, folliculitis decalvans, and tufted folliculitis.
METHODS: We conducted prospective and blinded histopathologic evaluation of clinically typical primary cicatricial alopecias. Biopsy specimens were taken from early affected scalp lesions and paired with samples from clinically unaffected areas in the same patient.
RESULTS: The lymphocytic and neutrophilic groups were readily distinguished histologically. However, within the two groups clinically distinct primary cicatricial alopecias could not be distinguished with current histopathologic techniques.
CONCLUSION: A descriptive, standardized histopathologic reporting of follicular architecture, type, location, and extent of the inflammatory infiltrate, and presence or absence of sebaceous glands, may be of greatest value in guiding the treatment of patients with primary cicatricial alopecias.VARIANTS ACNE KELOIDIALIS Acne keloidalis is a form of primary scarring alopecia.
Sperling LC, Homoky C, Pratt L, Sau P.
Department of Dermatology, Uniformed Services University, Bethesda, MD 20814, USA.
Arch Dermatol 2000 Apr;136(4):479-84 Abstract quote
OBJECTIVE: To better define the pathogenesis of acne keloidalis (AK).
DESIGN: Prospective, blinded study of histologic material collected from 10 patients with clinically typical AK.
SETTING: Outpatient dermatology clinic of a military tertiary care medical center.PATIENTS: Ten male volunteers 18 years or older with early AK lesions (1- to 4-mm firm papules on the lower occipital/nuchal region).
DATA SOURCE: Biopsy specimens from small, early lesions and from clinically uninvolved skin, studied histologically with transverse sectioning.
INTERVENTION: Three separate 4-mm punch biopsy specimens of the scalp (lesional, perilesional, and "normal" scalp) were obtained from each volunteer. The specimens were processed using transverse sectioning.
MAIN OUTCOME MEASURES: The primary variables for data analysis were the presence or absence of the following histologic features: premature loss of the inner root sheath; eccentric placement of shaft, with thinning of the outer root sheath; lamellar fibroplasia surrounding the follicle; loss of sebaceous glands; evidence of follicular destruction or scarring; inflammation; and intrafollicular or perifollicular microorganisms. The number and type of hairs were also recorded.
RESULTS: The most common findings in the 19 histologically abnormal specimens were perifollicular, chronic (lymphocytic and plasmacytic) inflammation, most intense at the level of the isthmus and lower infundibulum; lamellar fibroplasia, most marked at the level of the isthmus; complete disappearance of sebaceous glands, associated with inflamed or destroyed follicles; thinning of the follicular epithelium, most marked at the level of the isthmus; and total epithelial destruction (superficial and deep), with residual "naked" hair fragments. Even some "normal" specimens contained true follicular scars, demonstrating that normal-appearing scalp skin had previously been affected by the disease.
CONCLUSIONS: Acne keloidalis is a primary form of scarring alopecia, and many of the histologic findings closely resemble those found in certain other forms of cicatricial alopecia. Extensive subclinical disease may be present in patients with AK and can account for some of the permanent hair loss. Overgrowth of microorganisms does not appear to play an important role in the pathogenesis of the disease. There is no etiologic relationship between AK and pseudofolliculitis barbae. Therapies found to be useful in other forms of inflammatory scarring alopecia are useful in the treatment of early AK.
FOLLICULITIS DECALVANS
Immunopathogenesis of folliculitis decalvans: clues in early lesions.Department of Dermatological Sciences, University of Florence, Florence, Italy.
Am J Clin Pathol. 2008 Oct;130(4):526-34. Abstract quote
Folliculitis decalvans (FD) is a rare variant of primary cicatricial alopecia, for which the etiopathogenesis remains unclear.
Our purpose was to evaluate whether certain immunologic mechanisms might have a significant role in the pathogenesis of FD.Lesional scalp biopsy specimens from 7 patients with FD, 7 with lichen planopilaris, and 4 with alopecia areata were studied immunohistochemically by using monoclonal antibodies to CD1a, CD3, CD4, CD8, CD20, CD25, HLA-DR, interleukin (IL)-1beta, IL-4, IL-8, interferon gamma, tumor necrosis factor alpha, basic fibroblast growth factor (b-FGF), transforming growth factor (TGF)-beta, endothelial leukocyte adhesion molecule 1, intercellular adhesion molecule (ICAM)-1, and vascular cell adhesion molecule.
We showed that early FD lesions are characterized by an infiltration of activated T-helper cells, featuring mixed TH1/TH2 polarization. IL-8 and ICAM-1 may contribute to the infiltration of neutrophils, whereas b-FGF and TGF-beta may represent important mediators of the fibrosis that characterizes late-phase FD.FOLLICULAR DEGNERATION SYNDROME (Hot comb alopecia) Occurs on the crown of the scalp, usually in black women. It was once thought to be secondary to mechanical trauma but this has not been shown to be a consistent association. Histopathologically, there is premature desquamation of the inner root sheath below the level of the isthmus. The follicular degeneration syndrome in black patients. 'Hot comb alopecia' revisited and revised.
Sperling LC, Sau P.
Dermatology Service, Walter Reed Army Medical Center, Washington, DC.
Arch Dermatol 1992 Jan;128(1):68-74 Abstract quote
BACKGROUND--The history, physical examination, and histologic findings in 10 black women with a common, distinctive form of scarring alopecia (formerly called hot comb alopecia) were retrospectively studied. A detailed history of hair care habits was obtained, and scalp biopsy specimens were examined after both vertical and transverse sectioning.
OBSERVATIONS--Poor correlation is noted between the usage of a hot comb and the onset or progression of disease. The earliest observable histologic abnormality is the premature desquamation of the inner root sheath. In severely affected follicles this is followed by a chain of histologic events leading to complete follicular degeneration.
CONCLUSIONS--The term follicular degeneration syndrome (FDS) is proposed for this clinically and histologically distinct form of scarring alopecia. Historical information is incompatible with the hypothesis that hot comb usage causes the alopecia. It remains unclear whether the use of any of a variety of hair care products and techniques plays a role in the pathogenesis of this condition. Premature desquamation of the inner root sheath serves as a histologic marker for FDS follicular degeneration syndrome, and may be an important pathogenetic factor.
IDIOPATHIC Idiopathic scarring alopecia (Pseudopelade of Brocq, fibrosing alopecia, alopecia cicatrisata) Is essentially a diagnosis of exclusion. It usually occurs in women over 40 years old with a slow progressive course. There are footprints in the snow which may coalesce. Histopathologically, there is a scar. An elastic stain may be helpful identifying residual elastic fibers in the fibrous tracts. These fibers are not present in scarring alopecia secondary to lupus or lichen planus. POSTMENOPAUSAL FRONTAL FIBROSING ALOPECIA Is usually associated with perifollicular erythema. Histopathologically, there is considerable overlap with lichen planopilaris. Most investigators consider this disease to be a variant of this latter disorder. Postmenopausal frontal fibrosing alopecia. Scarring alopecia in a pattern distribution.
Kossard S.
Skin and Cancer Foundation, Sydney, Australia.
Arch Dermatol 1994 Jun;130(6):770-4 Abstract quote
BACKGROUND: Recession of the frontal hairline is a common event in postmenopausal women. This has been shown not to be a marker of gross androgenization, and is usually a progressive nonscarring alopecia. Six postmenopausal women, who developed a progressive frontal scarring alopecia, were studied and their clinical and laboratory data, as well as the results of scalp biopsy specimens in all six patients, were analyzed and compared with recognized forms of scarring alopecia and recently described findings in androgenetic alopecia.
OBSERVATIONS: The six postmenopausal women developed a progressive frontal hairline recession that was associated with perifollicular erythema within the marginal hairline, producing a frontal fibrosing alopecia extending to the temporal and parietal hair margins. Scalp biopsy specimens from the frontal hair margin showed perifollicular fibrosis and lymphocytic inflammation concentrated around the isthmus and infundibular areas of the follicles. Immunophenotyping of the lymphocytes showed a dominance of activated T-helper cells. Clinical review of all six cases showed a progressive marginal alopecia without the typical multifocal areas of involvement seen in lichen planopilaris or pseudopelade. None of the patients had mucous membrane or skin lesions typical of lichen planus. Hormonal studies, in five patients, showed no elevated androgen abnormalities.
CONCLUSIONS: Progressive frontal recession in postmenopausal women may show clinical features of a fibrosing alopecia. The histologic findings are indistinguishable from those seen in lichen planopilaris. However, the absence of associated lesions of lichen planus in all six women raises the possibility that this mode of follicular destruction represents a reaction pattern triggered by the events underlying postmenopausal frontal hairline recession.
Postmenopausal frontal fibrosing alopecia: a frontal variant of lichen planopilaris.
Kossard S, Lee MS, Wilkinson B.
Skin and Cancer Foundation Australia, NSW, Australia.
J Am Acad Dermatol 1997 Jan;36(1):59-66 Abstract quote
BACKGROUND: Lichen planopilaris usually produces multifocal areas of scarring alopecia. Recently, a condition in postmenopausal women characterized by progressive frontal hairline recession associated with scarring has been described.
OBJECTIVE: Our purpose was to study the clinical and histopathologic features and results of treatment in a group of women with the frontal variant of lichen planopilaris and to compare the immunohistochemical profile of scalp biopsy specimens from this subset with that found in the multifocal variant of lichen planopilaris.
METHOD: The clinical data as well as the histopathologic findings in 16 women with frontal fibrosing alopecia were collated. The immunohistochemical profile of six scalp biopsy specimens from the frontal hairline were compared with six specimens from women with multifocal lichen planopilaris.
RESULTS: In addition to the progressive frontal fibrosing alopecia in all 16 women, total loss or a marked decrease of the eyebrows was observed in 13. No evidence of lichen planus was observed at other sites. In one patient multifocal areas of lichen planopilaris developed in the scalp. The frontal fibrosing alopecia was slowly progressive but has stabilized in five patients. Biopsy specimens from the frontal hairline showed histologic changes identical to lichen planopilaris. Immunophenotyping failed to reveal any significant differences between the frontal and multifocal variants. No effective treatments emerged although oral steroids and antimalarials may temporarily slow the course. Hormone replacement therapy did not appear to influence the course of the alopecia.
CONCLUSION: Progressive frontal fibrosing alopecia is a clinically distinct variant of lichen planopilaris that affects in particular elderly women and frequently involves the eyebrows. The basis for this lichenoid tissue reaction targeting frontal scalp follicles and eyebrows is unknown.
PSORIASIS Psoriatic scarring alopecia: observations in four patients.
Bardazzi F, Fanti PA, Orlandi C, Chieregato C, Misciali C.
Department of Clinical and Experimental Medicine, University of Bologna, Italy.
Int J Dermatol 1999 Oct;38(10):765-8 Abstract quote
BACKGROUND AND DESIGN: Although acute or chronic hair loss in psoriasis of the scalp can be a symptom of the disease, until now there has been no agreement as to whether or not it is only restricted to erythrodermic, generalized pustular and scalp plaque psoriasis. The purpose of this study was to evaluate patients with chronic scalp psoriasis and alopecia and to determine if the alopecia was of the scarring type.
RESULTS: We report four cases of psoriatic scarring alopecia and describe the clinical and histologic features of these patients. The diagnosis was made by scalp biopsies (vertical and transverse sections) and other causes of scarring alopecia were excluded.
CONCLUSIONS: Psoriasis can cause scarring alopecia.
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Footprints in the snow-Term describing the characteristic bald spots caused by idiopathic scarring alopecia.
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