Follicular Degeneration Syndrome Research Articles

Central centrifugal cicatricial alopecia

Central centrifugal cicatricial alopecia is a common cause of progressive permanent apical alopecia. This unique form of alopecia includes entities previously know as “hot comb alopecia,” “follicular degeneration syndrome,” “pseudopelade” in African Americans and “central elliptical pseudopelade” in Caucasians. The etiology appears to be multifactorial and the condition occurs in all races.

Central centrifugal cicatricial alopecia: possible familial aetiology in two African families from South Africa

Central centrifugal cicatricial alopecia: possible familial aetiology in two African families from South Africa

Central centrifugal scarring alopecia as a rare form of scarring alopecia

The authors describe a rare form of scarring alopecia - central centrifugal scarring alopecia (synonym: follicular degeneration syndrome). A particular feature of this disorder is a combination of scarring alopecia with pathology of the thyroid gland. The authors also provide a classification of primary scarring alopecias.

Survivin, p53, MAC, Complement/C3, fibrinogen and HLA-ABC within hair follicles in central and centrifugal cicatricial alopecia

Context:Central centrifugal cicatricial alopecia (CCCA; originally entitled follicular degeneration syndrome, or hot comb alopecia) was first described in African American women utilizing hot combs and/or strong chemical hair care products.Case Report:A 67 year old African American female was evaluated for the presence of alopecic areas occurring on the scalp vertex, and spreading centrifugally. The alopecic lesions appeared as diffuse patches, including atrophic small areas surrounding individual hair follicles.Patients and Methods:Skin biopsies for hematoxylin and eosin examination, as well as for direct immunofluorescence and immunohistochemistry analyses were performed.Results:hematoxylin and eosin staining demonstrated histopathologic findings of premature desquamation of the inner root sheath and eccentric thinning of the follicular epithelium, supporting the diagnosis of CCCA. Direct immunofluorescence revealed strong depositions of Complement/C3, fibrinogen and kappa light chains around the hair follicles. Immunohistochemistry demonstrated increased expressions of HLA-ABC (as in African American patients with insulin independent diabetes mellitus). We also detected positive p53, bcl-2 and MAC staining in the hair follicle areas.Conclusions:Follicular degeneration syndrome may have an important immunological component previously not described, and multicolor immunofluorescence may be useful in establishing an early diagnosis.

Follicular streamers (stelae) in scarring and non‐scarring alopecia

Follicular streamers are residual fibrovascular tracts representing the impermanent lower third of the hair follicle below the bulge region. Streamers are generally not counted in transverse alopecia samples as they may represent catagen/telogen (CT) follicles and vellus-like (VL) follicles, or be mistaken as follicular scars. We evaluated 22 non-scarring alopecia cases, including alopecia areata (AA) and androgenetic alopecia (AGA), and 22 scarring alopecia cases, including follicular degeneration syndrome (FDS)/central centrifugal cicatricial alopecia and other scarring alopecia (OSA) disorders. We counted terminal follicular streamers found at a deep dermal level (L2) and followed them into a mid-dermal level at the central follicular unit (FU) to determine their precise derivation. We found streamers in 8/9 AA, 11/13 AGA, 6/12 FDS and 3/10 OSA cases. We counted a total of 74 streamers at L2, including 61 in non-scarring alopecia cases (p < 0.001). At the more superficial FU level, 72% of streamers corresponded to CT follicles, 25% to VL follicles and 3% to follicular scars. Follicular streamers are found predominantly in non-scarring alopecia cases. Streamers found at deep dermal or subcutaneous levels should be followed and identified at the FU level in order to obtain accurate follicular counts and follicular ratios needed for non-scarring alopecia diagnosis.

A comparison of vertical versus transverse sections in the evaluation of alopecia biopsy specimens

Both vertical and transverse sections are used in the histologic interpretation of alopecia biopsy specimens. Although a combination of the two may be optimal, the pathologist is frequently only provided with a single specimen. Even though the trend in recent years has been toward transverse sections in this setting, we are not aware of any published data directly comparing the two methods. One hundred two consecutive archived hair biopsy accessions that demonstrated comparable vertical and transverse sections were examined twice, each time in a random order. The pathologist's interpretation based only on the vertical sections and an interpretation based only on the transverse sections were compared with the original biopsy report, which had been based on the combination of vertical and transverse sections. In 76 cases, all 3 diagnoses were concordant (ie, the diagnosis made with vertical sections alone, the diagnosis made with transverse sections alone, and the original diagnosis were all in agreement). In 2 cases, neither the diagnosis made with vertical sections alone nor the diagnosis made with transverse sections alone were in full agreement with the original diagnosis. In 20 cases, only the diagnosis made with vertical sections was concordant with the original diagnosis. In 4 cases, only the diagnosis made with transverse sections alone was concordant with the original diagnosis. Our practice is heavily weighted toward scarring alopecia, and the results of our study may not be applicable to practices weighted toward other forms of alopecia. Because the cases had been signed out over a period of several years, the nomenclature for some entities changed. For the purposes of our study, we counted the diagnoses of follicular degeneration syndrome and idiopathic pseudopelade to be subtypes of (and concordant with) a diagnosis of central centrifugal cicatricial alopecia. In some cases, a definitive diagnosis was not possible at the time of the original diagnosis, but rather the pathologist had provided a histologic description and a differential diagnosis. For purposes of this study, an interpretation was considered to be concordant with the original descriptive diagnosis if all of the important histologic features were identified that had been described in the original report. Sampling error could have contributed to discordant diagnoses, but would be expected to affect both vertical and transverse samples in a random manner. The combination of vertical and transverse sections is superior to either alone. Although transverse sections have revolutionized the evaluation of alopecia, in this study, the diagnosis made with vertical sections alone had a higher concordance rate with the combination than did transverse sections alone. As there are advantages and disadvantages inherent in either method, when only a single biopsy specimen is submitted, it may be sectioned either vertically or transversely, at the discretion of the pathologist. With either method, serial step sections should be obtained to reduce the risk of missing important histologic findings.

Scarring alopecia and the dermatopathologist.

The evaluation of patients with cicatricial alopecia is particularly challenging, and dermatopathologists receive little training in the interpretation of scalp biopsy specimens. Accurate interpretation of specimens from patients with hair disease requires both qualitative (morphology of follicles, inflammation, fibrosis, etc.) and quantitative (size, number, follicular phase) information. Much of this data can only be obtained from transverse sections. In most cases, good clinical/pathologic correlation is required, and so clinicians should be expected to provide demographic information as well as a brief description of the pattern of hair loss and a clinical differential diagnosis. The criteria used to classify the various forms of cicatricial alopecia are relatively imprecise, and so classification is controversial and in a state of evolution. There are five fairly distinctive forms of cicatricial alopecia: 1) chronic, cutaneous lupus erythematosus (discoid LE); 2) lichen planopilaris; 3) dissecting cellulitis (perifolliculitis abscedens et suffodiens); 4) acne keloidalis; and 5) central, centrifugal scarring alopecia (follicular degeneration syndrome, folliculitis decalvans, pseudopelade). Not all patients with cicatricial alopecia can be confidently assigned to one of these five entities, and "cicatricial alopecia, unclassified" would be an appropriate label for such cases. The histologic features of five forms of cicatricial alopecia are reviewed. Dermatopathologists can utilize a "checklist" to catalog the diagnostic features of scalp biopsy specimens. In many, but not all, cases the information thus acquired will "match" the clinical and histologic characteristics of a form of cicatricial alopecia. However, because of histologic and clinical overlap between the forms of cicatricial alopecia, a definitive diagnosis cannot always be rendered.

Elastic tissue in scars and alopecia.

A recent report suggests that elastic fibers appear in scars in a time-dependent fashion. This observation prompted our investigation, because we have found elastic tissue stains helpful in determining the pattern of scarring in cases of permanent alopecia. We carried out this investigation to determine if the Verhoeff-Van Gieson (VVG) elastic stain can reliably differentiate scarred from non-scarred dermis and to test our hypothesis that elastic stained sections are helpful in distinguishing lichen planopilaris (LPP) from lupus erythematosus (LE), central progressive alopecia in black females ("follicular degeneration syndrome" and "hot comb alopecia" are other terms used to describe this condition) and classic ivory white idiopathic pseudopelade. We studied histological sections from surgical scars of known duration, stained with the VVG elastic stain and VVG-stained sections of scalp biopsies from patients with established lesions of permanent alopecia. In most cases, both vertical and transverse sections were examined. In every case, the VVG stain clearly differentiated scar from the normal surrounding dermis. Distinct patterns of elastic tissue allowed for correct classification in most of the well-established cases of permanent alopecia studied. We determined that the Verhoeff-Van Gieson stain is an excellent stain to evaluate the pattern of scarring in cases of permanent alopecia and elastic tissue stains may be helpful in the histological evaluation of alopecia.

Acquired scalp alopecia. Part II: A review.

The neutrophil-associated and infiltrative scarring alopecias are reviewed including folliculitis decalvans, tufted folliculitis, dissecting cellulitis of the scalp, acne keloidalis and follicular degeneration syndrome. The management of acquired scalp alopecia is also reviewed including newer, promising therapies. More specific agents targeting components of the androgen system will make the treatment of androgenetic alopecia more rewarding. Similarly new immunomodulatory therapies show great promise for the lymphocyte-associated alopecias and include a new generation of macrolide immunosuppressives (tacrolimus, SDZ ASM 981, and SDZ 281-240), some of which appear to have good transcutaneous absorption.

Vertical and transverse sections of alopecia biopsy specimens: Combining the two to maximize diagnostic yield

Background: Traditional vertical sections of scalp biopsy specimens contain few hair follicles. For this reason transverse sections of scalp biopsy specimens have been advocated. Both methods have advantages and disadvantages. We have developed a simple method that we believe offers the best of both methods. Objective: Our purpose was to assess the impact of combining vertical and transverse sections of scalp biopsy specimens. Methods: Two 4 mm punch biopsies are performed. One specimen is bisected vertically: half for hematoxylin-eosin (H-E) staining, half for direct immunofluorescence. The second specimen is bisected transversely and submitted for H-E. The three pieces of tissue for H-E staining are embedded in a single cassette. Results: Because a biopsy specimen for direct immunofluorescence is commonly obtained in cases of alopecia, our method does not add a surgical procedure. All three pieces of tissue for H-E staining are embedded in a single paraffin block. Therefore the cost of histologic interpretation is not increased. Our diagnostic yield improved. Transverse sections were superior in cases of lupus erythematosus and lichen planopilaris with focal follicular involvement. Features of the follicular degeneration syndrome were also best demonstrated in transverse sections. Interface changes, lupus panniculitis, miniaturized hairs, and trichomalacia were better demonstrated in vertical sections. Conclusion: Our method exploits the advantages of both vertical and transverse sections and improves diagnostic yield without increasing cost.

Follicular Degeneration Syndrome in Men

Follicular degeneration syndrome (FDS, formerly called hot comb alopecia) has only been described in black women. The clinical and histologic features of eight black men with a scarring alopecia resembling FDS were studied. All eight men had evidence of scarring alopecia, which was most prominent on the crown of the scalp. None of the men were using chemical or physical modalities to straighten or style the hair. Overall, this group of men had more evidence of active inflammation than did the previously described women with FDS. The histologic features in the men were identical to those found in women with FDS. The presence of premature desquamation of the inner root sheath and migration of the hair shaft through the outer root sheath serve as histologic markers of FDS and separate it histologically from other forms of scarring alopecia. Follicular degeneration syndrome is a common form of scarring alopecia in black men, just as it is in black women. In men, there is no association between chemical or mechanical hair styling techniques (eg, the "hot comb") and onset, progression, or severity of disease. The histologic features of FDS in men are identical to those in women.

Follicular degeneration syndrome in men

Follicular degeneration syndrome in men

The Follicular Degeneration Syndrome in Black Patients

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. 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. 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.

The follicular degeneration syndrome in black patients. 'Hot comb alopecia' revisited and revised

The follicular degeneration syndrome in black patients. 'Hot comb alopecia' revisited and revised