Rapidly progressive diffuse fibrosing alopecia

Du, Xuewen; Li, Zhongming; Zhu, Qilin; Xu, Wengrong; Li, Yuqian; Zhu, Jing; Elston, Dirk M.

doi:10.1016/j.jdcr.2019.07.025

Cited by 4 publications

(9 citation statements)

References 9 publications

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“…Each of these entities may show interface dermatitis involving follicular epithelium and concentric fibrosing around the isthmus and infundibulum, and we favor the term fibrosing alopecia to refer to these entities as a group. [1][2][3] Here we describe 5 patients with focal and diffuse fibrosing alopecia with overlapping features and a slowly progressive course lending further support to the concept that these diseases exist along a spectrum.…”

Section: Introductionsupporting

confidence: 70%

“…We proposed the term fibrosing alopecia to refer to the spectrum of cicatricial entities that share lichenoid folliculitis and fibrosis that mainly involves the upper portions of the follicle. 2 The current series of patients presented with slowly progressive alopecia that shared overlapping features. In the 3 younger patients, the hair thinning was more obvious at the hairline and relatively mild on the crown, whereas the 2 older patients had advanced hair thinning at the hairline as well as the crown.…”

Section: Discussionmentioning

confidence: 87%

“…Entities that share lichenoid folliculitis and concentric fibrosis around the isthmus and infundibulum include FFA, LPP, LPP, LPPDP, FAPD, and CPHL. [1][2][3] LPP presents as patchy hair loss, FFA involves a band-like area of the hairline, whereas CPHL and FAPD both involve androgendependent areas. 9 Although each has characteristic features, there exists a wide overlap between these entities and our series of patients suggesting they may belong to a common spectrum of disease.…”

Section: Discussionmentioning

confidence: 99%

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Focal and diffuse fibrosing alopecias: Classical lichen planopilaris, frontal fibrosing alopecia, fibrosing alopecia with a pattern distribution, cicatricial pattern hair loss, and lichen planopilaris diffuse pattern

Zhu

et al. 2020

JAAD Case Reports

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Section: Introductionsupporting

confidence: 70%

Section: Discussionmentioning

confidence: 87%

Section: Discussionmentioning

confidence: 99%

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Focal and diffuse fibrosing alopecias: Classical lichen planopilaris, frontal fibrosing alopecia, fibrosing alopecia with a pattern distribution, cicatricial pattern hair loss, and lichen planopilaris diffuse pattern

Zhu

et al. 2020

JAAD Case Reports

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“…Later, Du, et al . proposed the unifying concept ‘fibrosing alopecia’ to refer to the LPP subgroup of diseases, which share the histological features of lichenoid inflammation and fibrosis involving the distal HF 34 . Their rationale for lumping rather than splitting is that the aetiology and pathogenesis of these individual cicatricial entities have not been defined.…”

Section: Discussionmentioning

confidence: 99%

“…Later, Du, et al proposed the unifying concept 'fibrosing alopecia' to refer to the LPP subgroup of diseases, which share the histological features of lichenoid inflammation and fibrosis involving the distal HF. 34 Their rationale for lumping rather than splitting is that the aetiology and pathogenesis of these individual cicatricial entities have not been defined. Whilst we agree that there may be little utility in separate classifications at this stage, greater awareness of FAPD, either as a clinical entity in its own right or a variant of lichenoid folliculitis/fibrosing alopecia, will enhance distinction from its non-scarring mimics, AGA or AGA and concomitant seborrheic dermatitis.…”

Section: Discussionmentioning

confidence: 99%

Clinicopathological characteristics and treatment outcomes of fibrosing alopecia in a pattern distribution: A retrospective cohort study

Jerjen

Pinczewski

Sinclair

et al. 2021

Acad Dermatol Venereol

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Background Fibrosing alopecia in a pattern distribution (FAPD) is a primary cicatricial alopecia considered a subtype of lichen planopilaris (LPP). Objective The clinical and histopathological features, and treatment response of 24 female patients with FAPD were evaluated. Methods Demographic, clinical, histopathological and treatment data of patients with FAPD were retrospectively collected. Results Twenty‐four women were included (mean age 60.7 years). The mean Lichen Planopilaris Activity Index score was 1.50, and the median Sinclair grade was 3. Twelve patients had diffuse alopecia in the centroparietal region, four had frontal accentuation and eight had vertex accentuation of hair loss. Eight had associated loss of facial hair. Predominant trichoscopic features included hair shaft diameter variability (100%), perifollicular erythema and/or scaling (95%) and loss of follicular ostia (95%). Histopathological examination revealed features of scarring alopecia with interface changes in follicular epithelia mainly targeting terminal and intermediate hairs, and less frequently, vellus hairs. Treatment with hair growth‐promoting, anti‐inflammatory and anti‐androgen agents arrested disease progression in 14 patients and resulted in hair regrowth in two patients. Six patients had progression of their hair loss in spite of treatment. Limitations Referral bias to a specialist hair clinic, retrospective design and small sample size. Conclusions We present clinicopathological features of FAPD which can aid in the diagnosis of this insidious scarring alopecia. Our findings suggest a more favourable outcome if treatment is initiated in early stages of the disease.

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The spectrum of fibrosing alopecias

Orlando

Piraccini

Starace

2022

JEADV Clinical Practice

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Lichen planopilaris (LPP) is a primary lymphocytic cicatricial alopecia considered the most common cause of scarring hair loss in adults. It has been classified into three clinical variants: classical LPP, frontal fibrosing alopecia (FFA) and Graham Little-Piccardi-Lassueur syndrome. Classical LPP usually presents as a circumscribed cicatricial patch on the vertex and FFA as a band-like scarring alopecia of the frontotemporal hairline. Fibrosing alopecia in a pattern distribution (FAPD) and lichen planopilaris diffuse pattern (LPPDP) are two recently described subtypes of LPP that exhibit a diffuse involvement of the androgen-dependent region or the whole scalp, respectively. Classical LPP, FFA, FAPD and LPPDP share trichoscopic (cicatricial areas, absence of follicular openings, perifollicular erythema and hyperkeratosis) and histologic features (interface dermatitis involving follicular epithelium and concentric fibrosing around the isthmus and infundibulum).The remarkable clinical and histological overlap between these entities has led to the concept that these diseases exist along a spectrum. In the effort to identify classical LPP, FFA, FAPD and LPPDP with an umbrella term, we suggest to use 'lichenoid alopecias', as the distinguishing hallmark of these diseases is the lichenoid bandlike inflammatory infiltrate in the superficial dermis involving the infundibulum and isthmus of the hair follicle.

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Rapidly progressive diffuse fibrosing alopecia

Cited by 4 publications

References 9 publications

Focal and diffuse fibrosing alopecias: Classical lichen planopilaris, frontal fibrosing alopecia, fibrosing alopecia with a pattern distribution, cicatricial pattern hair loss, and lichen planopilaris diffuse pattern

Focal and diffuse fibrosing alopecias: Classical lichen planopilaris, frontal fibrosing alopecia, fibrosing alopecia with a pattern distribution, cicatricial pattern hair loss, and lichen planopilaris diffuse pattern

Clinicopathological characteristics and treatment outcomes of fibrosing alopecia in a pattern distribution: A retrospective cohort study

The spectrum of fibrosing alopecias

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