10.5826/dpc.1302a96
text/xml
2160-9381
2699
en
Riccardo Borroni
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
dermoscopy
Frontal Fibrosing Alopecia Associated with Oral Erosive Lichen Planus: Two Locations, One Disease
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-04-29
2022-07-12
2023-04-29
2023-06-05
e2023096
Dermatol Pract Concept
Riccardo Borroni
2023/04/29
10.5826/dpc.1302a96
e2023096
Dermatology Unit, Humanitas Research Hospital - IRCCS, Rozzano, MI, Italy
2160-9381
dermoscopy
en
e2023096
Dubin C, Glickman JW, Del Duca E, et al. Scalp and serum profiling of frontal fibrosing alopecia reveals scalp immune and fibrosis dysregulation with no systemic involvement. J Am Acad Dermatol. 2022;86(3):551-562. DOI: 10.1016/j.jaad.2021.05.016. PMID: 34044102.
Frontal Fibrosing Alopecia Associated with Oral Erosive Lichen Planus: Two Locations, One Disease
https://dpcj.org/index.php/dpc/article/download/2699/2068
   Frontal Fibrosing Alopecia Associated With Oral
  Erosive Lichen Planus: Two Locations, One Disease
          Francesco Toso1,2, Andrea Cortese1,2, Giovanni Fiorillo1, 2, Antonio Costanzo1,2,
                                     Riccardo G. Borroni1,2


1 Dermatology Unit, Humanitas Research Hospital - IRCCS, Rozzano, Milano, Italy
2 Department of Biomedical Sciences, Humanitas University, Pieve Emanuele, Milano, Italy



Key words: alopecia, lichen, dermopathology, dermoscopy
Citation: Toso F, Cortese A, Fiorillo G, Costanzo A, Borroni RG. Frontal Fibrosing Alopecia Associated with Oral Erosive Lichen Planus:
Two Locations, One Disease. Dermatol Pract Concept. 2023;13(2):e2023096. DOI: https://doi.org/10.5826/dpc.1302a96
Accepted: July 27, 2023; Published: April 2023
Copyright: ©2023 Toso et al. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: None.
Authorship: All authors have contributed significantly to this publication.
Corresponding Author: Francesco Toso, MD, Department of Biomedical Sciences, Humanitas University, Dermatology Unit, Humanitas
Research Hospital – IRCCS, Via Rita Levi Montalcini, 4 20090 Pieve Emanuele, Milano, Italy. E-mail: francesco.toso@humanitas.it




Introduction                                                             Wickham striae were also observed (Figure 1A). At the same
                                                                         time, a symmetrical eyebrow alopecia accompanied by a bi-
Frontal fibrosing alopecia (FFA) is a primary scarring alope-            lateral fronto-temporal hairline recession with loss of visible
cia that mainly affects postmenopausal women and is clini-               follicular ostia was noted (Figure 1B). Trichoscopic exam-
cally characterized by recession of the fronto-temporal line             ination showed rarefaction of follicular ostia, in the absence
with loss of the eyebrows [1].                                           of active inflammatory lesions, yellow dots, or vellus hair
                                                                         (Figure 1C). A clinical diagnosis of frontal fibrosing alope-

Case Presentation                                                        cia was then established. General examination excluded the
                                                                         presence of other lesions on the skin or concomitant lichen
An 89-year-old woman presented to our dermatology unit                   planopilaris. The patient laboratory tests were all within ref-
for a localized burning sensation in the oral cavity for 3               erence range except for mildly increased ferritin and mild
months, that was treated with oral acyclovir without notice-             neutrophilia. A comprehensive serological autoimmunity
able improvement. Family history included cutaneous lichen               panel gave negative results, while microscopic and culture
planus in her son. Personal history included arterial hyper-             examination of an oral cavity swab excluded the presence of
tension, osteoporosis, and rectal resection surgery for T2N0             bacteria or yeasts. Histopathologic examination of a biopsy
rectal adenocarcinoma. Physical examination revealed a few               specimen of the edge of an oral erosion showed a band-like
erosions, partially covered by whitish pseudo-membranes,                 lymphocytic infiltrate in the superficial chorion and at the
over an erythematous base on the oral vestibule and buc-                 junction with the overlying mucosa (Figure 1D), consistent
cal mucosa bilaterally, where whitish striae referable to                with the diagnosis of oral erosive lichen planus.




Research Letter | Dermatol Pract Concept. 2023;13(2):e2023096                                                                             1
                       Figure 1. (A) Erosions over an erythematous base, partially covered by whitish pseu-
                       domembranes, located at the level of the vestibule of the mouth and buccal mucosa.
                       (B) Symmetrical eyebrow alopecia accompanied by bilateral fronto-temporal hairline
                       recession. (C) Trichoscopy; loss of visible follicular ostia in the absence of active inflam-
                       matory lesions, yellow dots or vellus hair. (D) Histology; band-like lymphocytic infiltrate
                       in the superficial chorion and at the junction with the overlying mucosa.



Conclusions                                                            CD8+ T lymphocytes directed against cells in the basal layer
                                                                       of the epithelium, with an early increase in Th1-type cyto-
Frontal fibrosing alopecia belongs to the group of inter-              kines [3]. Similarly, in FFA the interaction of genetic, hor-
face (lichenoid) dermatitis, being characterized histopatho-           monal, and environmental factors would cause the loss of
logically by a perifollicular lymphohistiocytic infiltrate,            the immune privilege of hair follicles induced by Th1-type
resulting in fibrosis and scarring [1]. These features make            inflammation, with up-­regulation of the JAK/STAT signaling
FFA indistinguishable histologically from lichen planopi-              pathway and pro-fibrotic markers, increased production of
laris, of which it is therefore considered a clinical variant          IFN-γ, and subsequent activation of CD8+ lymphocytes di-
[1,2]. To date, only rare cases of FFA associated with oral            rected against the hair follicle [1,2]. Immune dysregulation
lichen planus (OLP) have been described [2]. Oral lichen               seems to be limited to the lesional skin [5].
planus is a chronic inflammatory disease also character-                    The coexistence of OLP and FFA in our patient suggests
ized by interface dermatitis on histopathology. Clinically,            that, although rarely associated, these two disorders could
it can be distinguished into three main subtypes: reticular,           represent two manifestations of the same immune-mediated
atrophic, and erosive [3]. It has been reported a small but            process. The presence of OLP should therefore be looked for
consistent increased risk of oral squamous cell carcinoma              in all patients with FFA by careful clinical inspection of the
development among OLP patients [4]. In OLP, stimulation                oral cavity, also taking into consideration the risk of malig-
of cell-mediated immunity leads to activation of cytotoxic             nant progression of oral mucosal lesions.




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Research Letter | Dermatol Pract Concept. 2023;13(2):e2023096                                                                         3
