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(a) Lateral view of frontal fibrosing alopecia Patient 1. Patient 1 had frontal hair loss with hypopigmentation scarring, erythema, scaling, and partial loss of eye brows. (b) Histopathology image from Patient 1. Mild perivascular and perifollicular lymphocytic infiltrate concentrated around the hair bulge area, dermal fibrosis, loss of sebaceous glands and follicular dropout. (c) Lateral view of frontal fibrosing alopecia Patient 2. Notice atrophic, hypopigmented alopecic skin along the frontal hair line that is affected by FFA. (d) Histopathology image from Patient 2. Mild perivascular and perifollicular lymphocytic infiltrate concentrated around the hair bulge area and perifollicular fibrosis
Source publication
Frontal fibrosing alopecia (FFA), a scarring type of alopecia, developed in two patients with a history of alopecia areata (AA). Both patients had biopsies to confirm this interesting series of pathology. Etiology and pathogenesis of this AA-to-FFA sequence will be discussed. © 2018 International Journal of Trichology | Published by Wolters Kluwer...
Contexts in source publication
Context 1
... sequence of pathology provides an opportunity to discuss the etiology that may be contributing to these cases. According to the biopsy reports, our two patients had lymphocyte-mediated scarring alopecia with mild perivascular and perifollicular lymphocytic infiltrates concentrated around the hair bulge area, characteristic of FFA [ Figure 1b and d]. [3] Patient 1 had patchy-patterned AA, affecting 50% of the scalp that responded to intralesional Kenalog and topical steroid treatment before FFA developed [ Figure 1a]. ...
Context 2
... to the biopsy reports, our two patients had lymphocyte-mediated scarring alopecia with mild perivascular and perifollicular lymphocytic infiltrates concentrated around the hair bulge area, characteristic of FFA [ Figure 1b and d]. [3] Patient 1 had patchy-patterned AA, affecting 50% of the scalp that responded to intralesional Kenalog and topical steroid treatment before FFA developed [ Figure 1a]. Patient 2 presented with ophiasis, loss of follicles, mild erythema, scaling, with partial loss of eyebrows, and eyelashes. ...
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Cicatricial (scarring) alopecia may result from perifollicular inflammation that destroys hair follicles and replaces them with connective tissue (primary cicatricial alopecia) or from damage done to follicles by other pathology that is not primarily directed against follicles (secondary cicatricial alopecia). Examples of such pathologies are: cuta...
Alopecia in the scalp region leads to psychosocial embarrassment for an individual. Alopecia could be due to several reasons, including genetic, hormonal, traumatic and infections. Cicatricial alopecias (CAs) are considered as trichological emergency, since their progression is rapid and always results in permanent hair loss. The pathogenesis, dise...
Frontal fibrosing alopecia is characterized by the presence of a lymphocytic inflammatory infiltrate around the upper follicle and by perifollicular fibrosis, which results in the destruction of the hair follicle. Recent reports have also found the presence of those findings in clinically unaffected areas. The aim of this report is to perform a dee...
Citations
... The absence of classic features for scarring alopecia in early cases of FFA without apparent alopecia of the hairline may have been misdiagnosed as AGA [56]. FFA-like cutaneous lupus erythematosus cases as well as association of FFA and alopecia areata in some patients were also described [57,58]. The FFA lesions are characterized by a lymphocytic infiltrate, perifollicular fibrosis and loss of follicles. ...
Frontal fibrosing alopecia (FFA) is recognized to represent a generalized process of inflammatory scarring alopecia. Apart from the classic form affecting the frontal hairline, there are a range of disease manifestations involving loss of eyebrows and of eyelashes, loss of peripheral body hair, fibrosing alopecia in a pattern distribution, facial and extrafacial skin, mucous membrane, and nail involvement. Classic linear, diffuse "zigzag", pseudo "fringe sign", androgenetic alopecia-like, cockade-like, ophiasis-like and incomplete patterns are distinguished. The aetiology of FFA remains obscure, but a number of pathogenetic hypotheses and treatments to halt disease progression have been proposed.
... As a result, LPP patients who have lost their IP at the bulge region may also be affected by AA, which is identified by the loss of IP at the bulb region, or vice versa. 15 In this study, we present a case of AA that consequently developed LPP in the remaining hair. To the best of our knowledge, there have been no reports of severe AA with a subsequent development of LPP in the remaining hair. ...
Although the coexistence of alopecia areata and lichen planopilaris is rare, if alopecic patches appear abruptly, this possible association should be kept in mind. Although the coexistence of alopecia areata and lichen planopilaris is rare, if alopecic patches appear abruptly, this possible association should be kept in mind.
... Previous reports of this association described two patients presenting FFA with past history of AA. The authors hypothesized that patients who have once lost IP at the bulb, are again prone to IP loss at the bulge later in life through the same mechanism [6]. To our knowledge, the concomitant association of FFA and AA has not been reported previously. ...
Background
Lonely hair sign is considered as a clue to the diagnosis of frontal fibrosing alopecia (FFA).
Objective
To report an undescribed variant of alopecia areata (AA) with which the patient developed single hairs and other features similar to FFA and to determine the underlying mechanism.
Methods
We conducted a prospective observational study in patients who presented with receding hairline and single hairs, evaluating the clinical, trichoscopic, and histological features and their correlation. Immunochemistry studies were performed to describe the microenvironment.
Results
Eighteen patients were enrolled in the study. Despite the similarity to FFA clinically, these patients showed different histopathology which revealed a normal number of pilosebaceous units, one anagen hair in one or more pilosebaceous units, and others in telogen stage, consistent with single hairs under the naked eye or under trichoscopy. The severity of the hair loss assessed by SALT was no more than 50, but the response to conventional therapy was poor.
Conclusions
This study reports a unique variant of AA. The pathological basis is an increase in the telogen hair follicles, with one anagen hair in one or more pilosebaceous units. Minimal inflammation consisting of CD3 ⁺ T lymphocytes and mast cells was demonstrated in the microenvironment.
Introduction
Scarring and non-scarring alopecias have rarely been described to occur together in the same patient. Distinguishing these two different types of alopecia is important as treatment and prognosis can be different.
Case presentation
Here, we report the first case of simultaneous alopecia areata (AA) and central centrifugal cicatricial alopecia (CCCA) in a 35-year-old woman. New alopecic patches were noted on her frontal and vertex scalp. Biopsy of the frontal scalp revealed miniaturized hair follicles and dense lymphocytic infiltrate surrounding the hair bulbs, consistent with AA; while biopsy of the vertex scalp revealed decreased hair follicles, perifollicular fibroplasia with eccentric atrophy of the follicular epithelium, and premature desquamation of the inner root sheath at the level of the lower isthmus, consistent with CCCA.
Discussion
Proposed mechanisms of these two alopecia types occurring together include loss of immune privilege, genetic predisposition, as well as unknown external factors that trigger an autoimmune lymphocytic response. Most recently, the peptidylarginine deiminase type III gene has been implicated in both diseases. Although treatment options can overlap between the two diseases, treatment response can differ and CCCA tends to have a worse prognosis.
Conclusion
Awareness of this concomitant presentation of two alopecic types is important for appropriate treatment and prognostication.
Despite patchy hair loss being typically observed in alopecia areata (AA), similar lesions can be seen in other forms of alopecia and the diagnosis is sometimes challenging. Of note, patchy primary scarring alopecia (SA) needs to be clearly distinguished from AA as SA can leave permanent hair loss. Herein, we report a previously unreported case of AA coexisting with SA successfully diagnosed by detailed trichoscopic investigation. A 42‐year‐old woman visited us with patchy hair loss lesions on the scalp. On physical examination, alopecic lesions sized up to 2 cm in diameter were observed in the right temporal and parietal regions. A gentle hair pull test collected dystrophic anagen hairs from some patches. Trichoscopy detected tapering hairs and black dots. The diagnosis of AA was made. However, some reddish patches were totally hair pull test negative, urging us to further evaluate the remaining lesions. Additional trichoscopic investigation revealed the disappearance of follicular ostia and the presence of a white and milky‐red area and peripilar scales, suggestive of SA. In histology, the clinically AA lesion showed peribulbar cell infiltration, while the potentially SA lesion demonstrated inflammatory cell infiltration around the isthmus and the decrease in hair follicles, some of which were replaced by fibrotic tissue. The final diagnosis of AA coexisting with SA was made. Intralesional corticosteroid injection improved AA but not SA. These findings emphasize the need for thorough trichoscopic examination for accurate diagnoses of rare hair loss conditions.
