10.5826/dpc.1304a229
text/xml
2160-9381
3247
en
Mariame  Meziane
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
phenotypic spectrum
Folliculitis Decalvans With Frontal Fibrosing Alopecia in a Dark Phototype: Presentation of Folliculitis Decalvans and Lichen Planopilaris Phenotypic Spectrum
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Research Letter
2023-10-31
2023-02-14
2023-10-31
2024-01-15
e2023229
Dermatol Pract Concept
Mariame  Meziane
2023/10/31
10.5826/dpc.1304a229
e2023229
Dermatology and Venereology Department, Ibn Sina Hospital, Mohammed V University of Rabat, Morocco
2160-9381
phenotypic spectrum
en
e2023229
Moreno-Arrones OM, Del Campo R, Saceda-Corralo D, Jimenez-Cauhe J, Ponce-Alonso M, Serrano-Villar S, Jaén P, Paoli J, Vañó-Galván S. Folliculitis decalvans microbiologic signature is specific for disease clinical phenotype. J Am Acad Dermatol. 2021 Nov;85(5):1355-1357.
Folliculitis Decalvans With Frontal Fibrosing Alopecia in a Dark Phototype: Presentation of Folliculitis Decalvans and Lichen Planopilaris Phenotypic Spectrum
https://dpcj.org/index.php/dpc/article/download/3247/2357
        Folliculitis Decalvans with Frontal Fibrosing
        Alopecia in a Dark Phototype: Presentation
      of Folliculitis Decalvans and Lichen Planopilaris
                     Phenotypic Spectrum
                  Basma Karrakchou1, Amani Fliti1, Amal El Fiboumi2, Fouad Kettani3,
                                Karima Senouci1, Mariame Meziane1


1 Dermatology and Venereology Department, Ibn Sina Hospital, Mohammed V University of Rabat, Morocco
2 Department of medicine, Moulay Youssef Hospital, Mohammed V University of Rabat, Morocco
3 Histopathology Center of the United Nations, Rabat, Morocco



Key words: folliculitis decalvans, frontal fibrosing alopecia, lichen planopilaris, phenotypic spectrum
Citation: Karrakchou B, Fliti A, El Fiboumi A, Kettani F, Senouci K, Meziane M. Folliculitis Decalvans with Frontal
Fibrosing Alopecia in a Dark Phototype: Presentation of Folliculitis Decalvans and Lichen Planopilaris Phenotypic Spectrum.
Dermatol Pract Concept. 2023;13(4):e2023229. DOI: https://doi.org/10.5826/dpc.1304a229
Accepted: April 13, 2023; Published: October 2023
Copyright: ©2023 Karrakchou 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: Basma Karrakchou: M.D. Dermatology and Venereology Department, Ibn Sina Hospital, Mohammed V University,
Rabat, Morocco. orcid number: https://orcid.org/0000-0001-5509-5412 E-mail adress: karrakchou.basma@gmail.com




Introduction                                                             Case Presentation
Lichen planopilaris (LPP) and folliculitis decalvans (FD) are            A 42-year-old premenopausal woman presented with a 10-year
two primary scarring alopecias recently associated in a phe-             history of vertex pustules and crusts leading to scarring
notypic spectrum in which they occur simultaneously or in                patches of alopecia. Nine years later, she presented pruritus in
a bi-phasic presentation, either in the same scalp location              the frontal hairline. The physical examination found a pho-
or in different scalp areas [1]. We report a sequential onset            totype V patient with two vertex keloid patches of alopecia
of vertex FD and frontal fibrosing alopecia (FFA), a variant             measuring 6 cm and 4 cm in diameter respectively, contain-
of LPP, as an exceptional presentation of this spectrum in               ing tufts, follicular pustules, hemorrhagic crusts, milky-red
phototype V.                                                             areas, and dilated vessels on trichoscopy (Figure 1, A and C).




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023229                                                                          1
        Figure 1. (A) Clinical aspect of folliculitis decalvans: 2 vertex patches of alopecia with tufted hairs, pustules and crusts on
        an underlying keloid scar. (B) Front view showing an associated frontal fibrosing alopecia, and lichen planus pigmentosus.
        (C) Vertex trichoscopy: pustules and hemorrhagic crusts surrounding tufts of more than 5 hairs. There were cicatricial
        milky-red areas with dilated vessels and no follicular openings. (D) Frontal hairline trichoscopy: perifollicular erythema,
        peripilar hyperkeratosis, and tubular hair casts. Absence of vellus hairs was noticed. Yellow dots were seen on dark photo-
        type and corresponded to sebaceous glands.




There was an associated 2.5 cm linear frontal hairline reces-             isotretinoin (0.2 mg/kg per day for at least 6 months), in-
sion with trichoscopy showing peripilar hyperkeratosis and                tralesional injections of corticosteroids every 6 weeks for
erythema, tubular hair casts, and yellow dots (Figure 1, B                FFA and FD, and topical fusidic acid twice a week for FD.
and D). No vellus hairs were seen. Additional eyebrow loss                Within one year of treatment, FFA stabilization was achieved
covered by micropigmentation, facial papules, and facial hy-              with no more peripilar erythema and hyperkeratosis, no
perpigmentation were noticed.                                             hair loss progression, and no hair regrowth. A subsidence
    A diagnosis of FD associated with FFA and lichen planus pig-          of keloid FD scars was obtained with persistence of some
mentosus was confirmed by histopathology (Figure 2, A-D).                 pustules, which needed the adjunction of oral azithromycin
No bacterial sample was taken from vertex patches of alope-               (500 mg per day, 3 days per week for 3 weeks) for remis-
cia due to ongoing oral doxycycline (50 mg twice a day) at                sion. Currently, the patient is still under treatment, with a
that time, with no improvement after 6 months. The decision               taper-off of isotretinoin dose after one year (0.1 mg/kg
was made to cease treatment and to prescribe oral low-dose                per day), in the view of its discontinuation.




2                                                                             Research Letter | Dermatol Pract Concept. 2023;13(4):e2023229
        Figure 2. (A, B) Histopathology view (Hematoxylin-Eosin stain, (A) low magnification, (B) high magnification) of vertex
        scalp biopsy showing a dense peripilar neutrophilic infiltrate, infundibular pustule, and plasma cell exocytosis. They de-
        struct focally hair shafts. Fibrosis is noted in deep dermis. (C, D) Histopathology aspect (Hematoxylin-Eosin stain, (C) low
        magnification, (D) high magnification) of frontal hairline scalp biopsy: isthmic and infundibular perifollicular hyperkera-
        tosis associated with a moderate dermic lymphocytic infiltrate and dense fibrosis.




Conclusions                                                             is then more or less a mixture of FFA activity signs, such as
                                                                        peripilar erythema and scaling, with FD activity patterns, as
FD and LPP are primary scarring alopecias recently com-                 crusts and pustules. In late stages of scarring alopecia, there
bined in the “FD and LPP phenotypic spectrum” (FDLPPPS)                 are milky-red areas and tufts in FD, and no follicular open-
[1-5]. To date, mainly vertex scalp LPP cases have been                 ings in FFA.
reported in association with FD [1-5]. To the best of our                   Histopathology is the hallmark of FDLPPPS’ final diag-
knowledge, only 2 observations described FFA within the                 nosis, especially when FD and LPP occur in the same area.
phenotypic spectrum, and the two patients were phototype                The condition is characterized by more follicular packs
II (Table 1) [1,5].                                                     and plasma cells exocytosis over neutrophils [2], and less
   FFA is prominent in postmenauposal light skinned                     ­bacterial infiltrate [8].
women. When there is additional lichen planus pigmentosus,                  Both conditions progress into scarring alopecia, and
as in our case, FFA mostly affects dark phototype females [6].          early diagnosis is then compulsory to an adequate manage-
FD generally occurs in African American young men. And                  ment based on anti-inflammatory agents (corticosteroids and
scalp keloid scars are described in FD only when associated             isotretinoin) associated with oral antibiotics [1-5]. However,
with acne keloidalis nuchae, which occurs in 21% [7].                   there is no consensus in FDLPPPS treatment, and the therapy
   Trichoscopy has a prominent place by showing either sep-             duration remains to be established. A long-term follow up is
arate features of FD and FFA, or a progressive switch from              also needed to manage the frequent disease flares occurring
one to another using dynamic trichoscopy [1]. The aspect                after treatment completion.




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023229                                                                          3
4
                                                                                                  Table 1. Literature reported cases of Frontal Fibrosing Alopecia associated with Folliculitis Decalvans
                                                                                                                                                                                                Bacterial
                                                                        Age (y),        Fitzpatrick                                                                                              culture                                               Reference
                                                                Patient Gender          phototype           Onset          Clinical aspect   Trichoscopic aspect          Histology           (scalp swab)        Treatment            Outcome             #
                                                                1          42, F       II               Concomitant Frontal hairline         Frontal trichoscopy:   FFA: peri-isthmic         MSSA           Lymecycline 408mg       Intermittent     [1] Case 9
                                                                                                                    recession and            peripilar              fibrosis and                             bd followed by oral     recurrences
                                                                                                                    vertex area of           hyperkeratosis and     lymphocytic infiltrate                   and topical fusidic     controled with
                                                                                                                    scarring alopecia.       erythema consistent    FD: Polytrichia,                         acid for 3 weeks,       intermittent
                                                                                                                                             with FFA.              perifollicular                           and oral zinc sulfate   topical and oral
                                                                                                                                             Vertex trichoscopy:    neutrophilic infiltrate                  125mg bd                fusidic acid
                                                                                                                                             tufts, crusts,         and microabces.                                                  and topical
                                                                                                                                             follicular plugging,                                                                    corticosteroids.
                                                                                                                                             and pustules
                                                                                                                                             compatible with FD.
                                                                2          65, F       II               Sequential: Pustular frontal   Frontal trichoscopy:         Frontal hairline        Sterile          Topical                 No disease        [5]
                                                                                                        FFA then FD hairline alopecia. peripilar                    scalp: Perifollicular                    corticosteroids for     flare occured
                                                                                                                                       hyperkeratosis               lymphocytic dermic                       1 month.                within 3
                                                                                                                                       erythema and                 infiltrate and presence                                          months of
                                                                                                                                       pustules.                    of plasma cells.                                                 follow up.
                                                                                                                                                                    Intraepidermal pustule
                                                                                                                                                                    with neutrophils were
                                                                                                                                                                    present.
                                                                Our        42,F        V                Sequential  Linear frontal           Frontal trichoscopy:   FFA: infundibular         Not            Doxycyclines 50mg       Stabilization
                                                                patient                                 FD then FFA hairline recession       peripilar              hyperkeratosis and        performed      bd followed by          of FFA,
                                                                                                                    and two vertex           hyperkeratosis,        dermic lymphocytic                       long course oral        subsidence of
                                                                                                                    keloid patches of        sliding sheaths,       infiltrate with                          isotretinoin 20mg/d     keloid patches
                                                                                                                    alopecia.                perifollicular         fibrosis.FD:                             and intralesional       of alopecia and
                                                                                                                                             erythema, and          multicoupound hair                       corticosteroids,        drying of FD
                                                                                                                                             absence of vellus      structures and plasma                    and topical fusidic     lesions within
                                                                                                                                             hairs.                 cells exocytose with                     acid. Additional        1 year.
                                                                                                                                             Vertex trichoscopy:    neutrophilic infiltrate                  oral azithromycin
                                                                                                                                             tufts, pustules, and   and dermic fibrosis.                     500mg/d for 3days
                                                                                                                                             crusts, associated                                              per week during
                                                                                                                                             with cicatricial                                                3 weeks were
                                                                                                                                             milky-red areas and                                             prescribed.
                                                                                                                                             dilated vessels.
                                                                FD = folliculitis decalvans; FFA = frontal fibrosing alopecia.




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023229
    In conclusion, this case highlights the possible emergence               report of two paediatric cases. J Eur Acad Dermatol Venereol.
of new variants within the FDLPPPS, as the occurrence of                     2021;35(10):e674-e676. DOI: 10.1111/jdv.17379. PMID:
                                                                             34014598.
FD-FFA in a dark phototype. The keloid evolution of FD
                                                                        4.   Zhang X, Zhu M, Zhou J, Wu S, Liu J, Qin Q. Folliculitis De-
lesions is possible without an associated acne keloidalis nu-
                                                                             calvans and Lichen Planopilaris Phenotypic Spectrum: A Case
chae in highly pigmented skins. FD and FFA have both a                       Report. Clin Cosmet Investig Dermatol. 2022;15:993-996.
scarring evolution, and trichoscopy allows an early diagno-                  DOI: 10.2147/CCID.S365566. PMID: 35677221. PMCID:
sis orientation for an urgent management. Further case re-                   PMC9167836.
ports are however needed to fully characterize the FDLPPPS              5.   Lobato-Berezo A, González-Farré M, Pujol RM. Pustular frontal
                                                                             fibrosing alopecia: a new variant within the folliculitis decal-
spectrum.
                                                                             vans and lichen planopilaris phenotypic spectrum? Br J Der-
                                                                             matol. 2022;186(5):905-907. DOI: 10.1111/bjd.20962. PMID:
References                                                                   34939665.
                                                                        6.   Pirmez R, Duque-Estrada B, Donati A, et al. Clinical and dermo-
 1. Yip L, Barrett TH, Harries MJ. Folliculitis decalvans and lichen         scopic features of lichen planus pigmentosus in 37 patients with
    planopilaris phenotypic spectrum: a case series of biphasic clin-        frontal fibrosing alopecia. Br J Dermatol. 2016;175(6):1387-
    ical presentation and theories on pathogenesis. Clin Exp Der-            1390. DOI: 10.1111/bjd.14722. PMID: 27233640.
    matol. 2020;45(1):63-72. DOI: 10.1111/ced.13989. PMID:              7.   Doche I, Coelho EQ, Quaresma MV, da Matta Rivitti-Machado
    31017678.                                                                MC. Acne keloidalis nuchae and folliculitis decalvans: same pro-
 2. Egger A, Stojadinovic O , Miteva M. Folliculitis Decalvans and           cess affecting the follicle or coexisting diseases? A retrospective
    Lichen Planopilaris Phenotypic Spectrum-A Series of 7 New                study. Int J Dermatol. 2019;58(10):e200-e203 DOI: 10.1111
    Cases With Focus on Histopathology. Am J Dermatopatho.l                  /ijd.14565. PMID: 31241169.
    2020;42 (3):173-177. DOI: 10.1097/DAD.0000000000001595.             8.   Moreno-Arrones OM, Del Campo R, Saceda-Corralo D, et al.
    PMID: 31855586.                                                          Folliculitis decalvans microbiologic signature is specific for dis-
 3. Ramos PM, Melo DF, Lemes LR, et al. Folliculitis decal-                  ease clinical phenotype. J Am Acad Dermatol. 2021;85(5):1355-
    vans and lichen planopilaris phenotypic spectrum: case                   1357. DOI: 10.1016/j.jaad.2020.10.073. PMID: 33144151.




Research Letter | Dermatol Pract Concept. 2023;13(4):e2023229                                                                                5
