前额纤维化性脱发二例

例1女,44岁,额颞部发际线后移4年,面部多发性肤色小丘疹2年.皮肤科检查:额颞部发际线后移,局部皮肤光滑菲薄,可见残存的细小毛发;眉毛、腋毛和阴毛部分脱落;额颞部、双下颌角处可见弥漫性分布许多粟粒大小的肤色小丘疹.皮肤镜下可见毛囊开口数减少,毛发直径不一,瘢痕性白斑和毛囊周围红斑.例2女,55岁,额颞部毛发稀少2年.皮肤科检查:双侧额颞部发际线后移,眉毛、腋毛和阴毛部分脱落.2例患者的组织病理检查均可见毛囊周围以淋巴细胞为主的浸润,基底细胞液化变性,毛囊周围有板层状纤维化.2例患者的临床和组织病理表现均符合前额纤维化性脱发的诊断.     

Frontal fibrosierende Alopezie Kossard

Within the group of cicatricial alopecias, Kossard first described frontal fibrosing alopecia (FFA) in 1994 as a variant of lichen planopilaris (LPP). This classification is based on the histopathological findings of FFA and LPP, which are identical and therefore not separable. The clinical picture of FFA, however, is very characteristic and marked by regionally distinct structures of the skin. Typically, postmenopausal women present with a band-shaped atrophy that is several centimeters wide located in the frontotemporal area. Adjacent to the hairline, perifollicular erythema and papules can be found. In the majority of patients there is a loss of the eyebrows. Circumscribed alopecia and perifollicular papules occur only rarely on the extremities or the trunk. Etiology and pathogenesis of FFA are unclear. Hormone-related involutionary phenomena of the follicle, genetic factors, disruption of lipid homeostasis, and in accordance with the lichen planus associated T?cell cytotoxic autoimmune response are discussed. Treatment of FFA is difficult. Atrophy cannot be influenced by the currently available treatment options. With regard to the follicular inflammation, topical steroids and systemic hydroxychloroquine, antiandrogens, and tetracyclines are mainly used on a topical basis.     

Dermoscopic findings in frontal fibrosing alopecia: report of four cases

Background  Frontal fibrosing alopecia (FFA) is characterized by frontotemporal hair recession and eyebrow loss and by a histopathology identical to lichen planopilaris. Differential diagnosis from other types of alopecia, including alopecia areata (AA), is necessary in some cases.
Objective  To describe dermoscopic findings of FFA and to investigate the possibility of utilizing dermoscopy as a diagnostic tool for FFA.
Methods  Four cases of FFA diagnosed by clinical and/or histological findings were examined by dermoscopy.
Results  The loss of orifices, perifollicular erythema or scale was seen in all the four cases (4/4), in three cases (3/4) or in two cases (2/4), respectively. None of the cases showed yellow dots characteristic of AA. Immunohistochemistry showed T lymphocyte infiltration into the infundibulum and isthmus.
Conclusion  Dermoscopy is a helpful diagnostic tool for FFA especially to distinguish it from AA.     

Diagnostic and therapeutic assessment of frontal fibrosing alopecia

Frontal fibrosing alopecia is a clinical entity characterized by recession of the frontotemporal hairline in middle-aged and older women. Since it was first described in 1994, more than 100 cases have been reported describing other clinical manifestations such as eyebrow and axillary alopecia, and perifollicular inflammation that help in the diagnosis of the disease and the differential diagnosis with other scarring alopecias. Histopathology reveals an inflammatory infiltrate and perifollicular lamellar fibrosis. Although numerous therapeutic options have been tested, including corticosteroids, finasteride, and minoxidil, none have shown clear benefits in terms of halting the progression of the alopecia.     

Clinical characteristics,trichoscopy, histopathology and treatment outcomes of frontal fibrosing alopecia in an Asian population: A retro-prospective cohort study

Frontal fibrosing alopecia (FFA) is a distinctive lymphocytic scarring alopecia with rapid increase in prevalence. Most FFA series are retrospectively reported from Caucasians with only few from Asians. The objective of this study was to characterize the clinical, trichoscopic and histopathological findings as well as treatment outcomes. This was a retro-prospective cohort study of patients diagnosed with FFA from 1 January 2010 to 1 November 2019. All patients were asked to present for re-examination. Clinical, trichoscopic, histopathological and laboratory data were recorded. A questionnaire was used to investigate hair care, hairstyle and facial skin care compared with age-matched normal controls. Multivariate analysis was performed in order to clarify factors associated with severity. All 58 FFA patients were female, of whom 27.6% were premenopausal, 37.7% had a history of surgical menopause, 13.8% had thyroid diseases, 69% had eyebrow loss and 32.8% facial papules. On physical examination, 10.3% showed linear pattern, 46.6% diffuse pattern and 43.1% pseudo-fringe sign. Concomitant lichen planopilaris was found in 25.9%, lichen planus pigmentosus in 24.1% and female pattern hair loss in 48.3%. The most common trichoscopic characteristics in the frontal hairline were lack of follicular ostia (91.4%), perifollicular scales (79.3%) and perifollicular erythema (63.8%). Up to 90% of patients reported FFA as improved or stable after receiving antiandrogen (finasteride or dutasteride) or antimalarial with topical treatment. Multivariate analyses revealed that facial lentiginous macules and trichoscopic perifollicular erythema at the frontal area were FFA severity-associated factors. “Front puff” Thai hairstyle was associated with FFA, while sunscreens and other cosmetic products were not. In conclusion, diffuse and pseudo-fringe sign pattern are common in Asian FFA. The most common autoimmune systemic comorbidity is thyroid disease, while common concomitant dermatological diseases are female pattern hair loss, lichen planopilaris and lichen planus pigmentosus. Antiandrogens or antimalarial plus topical treatment are the most useful therapy.     

Case series of familial frontal fibrosing alopecia and a review of the literature

Frontal fibrosing alopecia (FFA) is a distinctive form of scarring alopecia presenting with partial eyebrow loss and frontal temporal parietal recession of the hairline. Its etiology remains unknown, and there is no definitive treatment. Information in familial cases of FFA is scarce. We conducted a retrospective cohort study describing the mean clinical findings, treatment, and also the mean differences between premenopausal and postmenopausal cases of familiar FFA. Data analysis from case was performed on eight patients with a familiar history and diagnosis of FFA seen at the Alicante Aesthetic Dermatology Centre between January 2009 and June 2014. All patients in this cohort were females. Mean age at onset was 65 year (range 60–75) in the postmenopausal patients and 39 year (range 33–47) in the premenopausal women. All menopausal patients were in an advanced stage when the disease had already developed in the frontal and/or temporal parietal hairline region. However, the daughters, all of them premenopausal age, attended the consultation with mild involvement of the eyebrows in all four cases and mild impairment of the frontal hairline in three of them. Specific clinical findings in familial FFA are poorly communicated until nowadays although the number of familial cases arises until 8% in the main case series published in recent years. Early diagnosis in premenopausal stage is frequent in our case series and allows us to begin the protocol treatment in the first stage of the disease, but long‐term progression will remain uncertain until a definitive treatment could be established by multicenter randomized controlled trials.     

Frontal fibrosing alopecia update

Frontal fibrosing alopecia(FFA) is a recently described form of primary cicatricial alopecia, characterized by progressive recession of the frontotemporal hairline and eyebrow loss, occurring predominantly in postmenopausal women. The incidence of FFA has increased significantly during the last decade and we may be facing an epidemic of the disease. Because this condition causes permanent hair loss, prompt diagnosis and treatment are essential for obtaining optimal outcome. This article reviews existing knowledge on epidemiology, etiopathogenesis, clinico-histological features, diagnosis,and treatment modalities of FFA.     

Recalcitrant lichen planopilaris and frontal fibrosing alopecia responding to tildrakizumab

Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are lymphocytic, cicatricial alopecias. Clinically, LPP presents with multifocal patchy alopecia, while FFA, considered a variant of LPP, results in hairline recession. Frontal recession in FFA may progress as far as the mid‐scalp and infrequently beyond. Treatment to arrest the inflammatory process can be challenging and response variable. We report a case of recalcitrant lichen planopilaris and frontal fibrosing alopecia demonstrating significant clinical improvement after four doses of the interleukin‐23 monoclonal antibody tildrakizumab.     

Frontal fibrosing alopecia

Frontal fibrosing alopecia (FFA) is a patterned primary cicatricial alopecia that was first described in 1994. Once rare, the incidence of FFA has increased dramatically, representing the current most common cause of cicatricial alopecia worldwide. FFA typically begins in postmenopausal women with symmetrical, progressive recession of the frontotemporal hairline together with bilateral loss of the eyebrows. FFA has a distinctive clinical phenotype, which remains a challenge. The histology is identical to lichen planopilaris (LPP), but only a small number of patients have coincidental LPP, usually of the scalp. The vast majority of patients have no evidence of lichen planus elsewhere, and the symmetry and patterned nature of the hair loss are unusual for LPP. Familial cases of FFA are reported, and gene associations have been identified in population studies; however, the pathophysiology remains controversial. Without treatment, FFA is slowly progressive, and although many treatments have been prescribed, the response is often disappointing. We review the pathogenesis, epidemiology, clinical features, histology, and treatment of FFA.     

Postmenopausal frontal fibrosing alopecia

Recently a new entity, postmenopausal frontal fibrosing alopecia, was added to the established subtypes of scarring alopecias affecting postmenopausal women. This condition is characterized by a progressive frontal hairline recession associated with scarring. We studied the clinical and histopathologic features in four women with this disorder. Of note, a history of bilateral oophorectomy in two of them appears to be a new association. All four cases had frontoparietal recession of the hairline and two of them also had loss of their eyebrows. None of our four patients had any mucous membrane or other skin lesions. Histological examination showed perifollicular fibrosis and lymphocytic inflammation around the isthmus and infundibular areas of the follicles. No effective treatments have emerged for this type of postmenopausal alopecia, but progression of the hair loss and scarring appears to be self-limiting. We believe that this condition is a distinct clinicopathological variant of lichen planopilaris.     

Alopecia frontal fibrosante: estudio retrospectivo de 75 pacientes

Background and objectiveFrontal fibrosing alopecia is an increasingly common form of scarring alopecia. The aim of this study was to describe the demographic and clinical characteristics of patients with FFA seen at the trichology unit of a medium-sized regional hospital and to report on treatments used.Material and methodWe reviewed the medical records of all patients with FFA seen at the trichology unit of Hospital Universitario Infanta Sofía in Madrid, Spain between May 2016 and May 2018. We analyzed associations between disease severity, clinical patterns, need for oral medications, and other characteristics.ResultsSeventy-five patients (73 women and 2 men) were studied. Diagnosis was clinical in most cases and 13 cases (17.3%) were confirmed histologically. Median (interquartile range) age at reported onset of symptoms was 61 (12) years. Involvement of the eyebrows was recorded in 70 patients (93.3%) and signs of oral and genital lichen planus in 7 (9.6%). Eleven patients (14.7%) had hypothyroidism and 15 (20.0%) had signs of rosacea. Only 5 of the patients who presented a linear pattern (21.7%) had severe hairline recession. Patients with unstable and/or symptomatic disease (n = 24) were treated with oral medications (5-alpha reductase inhibitors, hydroxychloroquine, corticosteroids, and isotretinoin) or intralesional corticosteroids. Eighteen patients (75.0%) achieved disease stability. Ten of the 15 patients with signs of rosacea and 10 of those with facial papules required systemic treatment.ConclusionMost of the patients in this series of FFA were postmenopausal women. The prevalence of oral and genital lichen planus was higher than that observed in the general population. Patients with a linear pattern had less severe disease. Facial papules were more common in younger patients and both facial papules and rosacea were associated with a greater need for oral treatment.     

Alopecia areata masquerading as frontal fibrosing alopecia

SUMMARY Postmenopausal women with frontal recession may represent a diagnostic challenge, as frontal fibrosing alopecia and alopecia areata may be clinically difficult to distinguish. A 53-year-old postmenopausal woman presented with a progressive fronto-temporal marginal alopecia with sparing of her eyebrows. Scalp biopsy of the affected frontal hairline revealed peribulbar lymphocytic inflammation, but no evidence of lichenoid inflammation, perifollicular fibrosis or scarring. Whereas the pathology strongly favoured alopecia areata, the clinical features overlapped with frontal fibrosing alopecia, a variant of lichen planopilaris targeting the frontal scalp. This paper presents an atypical clinical presentation of alopecia areata, which may be mistaken for frontal fibrosing alopecia.     

Lichen planopilaris

ABSTRACT:   Lichen planopilaris is a chronic scarring alopecia characterized by follicular hyperkeratosis, perifollicular erythema, and loss of follicular orifices. The scalp lesions may be single or multiple and commonly involve the vertex and parietal area. The hair follicles at the margin of the alopecic patches reveal perifollicular erythema. Anagen hairs can be pulled out easily in active lesions. Associated cutaneous, nail, and mucous membrane lichen planus may be present. Commonly encountered symptoms and signs are increased hair shedding, itching, scaling, burning, and tenderness. Differentiation from other cicatricial alopecia can be performed through meticulous evaluation of the clinical, histopathologic, and immunohistopathologic findings. Treatment strategies depend on the disease activity and physician expertise. Although there are no definitive curative modalities, some new discoveries and conceptual advances continue to broaden our treatment options of this complex condition.     

Frontal fibrosing alopecia: a survey in 16 patients

BACKGROUND: Postmenopausal frontal fibrosing alopecia (PFFA) was described by Kossard et al. as a progressive recession of the frontal hairline affecting particularly postmenopausal women. Further cases of PFFA have been reported to date, all of them considering it as a variant of lichen planopilaris on the basis of its clinical, histological and immunohistochemical features. OBJECTIVE: To describe clinical features, and response to treatment of 16 cases of frontal fibrosing alopecia diagnosed at our department in the last 6 years. METHODS: In addition to clinical data, biopsies and laboratory tests (antinuclear antibodies, sex hormones, thyroid hormones) were performed in order to rule out other causes of scarring alopecia. Patients were treated with intralesional corticosteroids, finasteride, and minoxidil, depending on the stage of the disease and association to androgenetic alopecia. RESULTS: All patients presented progressive alopecia localized to the frontal and temporal hairlines. Eight patients (50%) had loss of eyebrows, and six patients (37.5%) had axillar alopecia. Ages ranged from 45 to 79. Three of these women were premenopausal. Androgenetic alopecia was evident in seven patients (43.8%). All patients biopsied showed perifollicular lymphocitic infiltrate with lamelar fibrosis limited to the upper portions of the follicle. The progression of the condition stopped in most patients after a variable period on treatment. When treatment was abandoned the alopecia progressed to 'clown alopecia' appearance. DISCUSSION: Cases of Kossard's type scarring alopecia affecting premenopausal women made us consider that this condition is not exclusive of postmenopausal women. Differential diagnosis should take into account conditions like female androgenetic alopecia, fibrosing alopecia in a pattern distribution, alopecia areata, and chronic lupus erythematosus. Except for the pattern of alopecia, lichen planopilaris and frontal fibrosing alopecia are indistinguishable, thus the latter is included as a variant of lichen planopilaris. Although the disease tends to spontaneous stabilization, intralesional and topical corticosteroids, and anti-androgens may stop the progression of the disease and improve the female androgenetic alopecia that usually is associated to FFA.     

“Normal-appearing” scalp areas are also affected in lichen planopilaris and frontal fibrosing alopecia: An observational histopathologic study of 40 patients

Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are lymphocyte-mediated scarring alopecias which clinically affect primarily the anterior and mid-scalp. However, unaffected scalp areas have not yet been investigated in a systemic manner. In this study, we assessed histopathologic changes in affected and unaffected scalp in both diseases and healthy control subjects and compared these findings with clinical signs and scalp symptoms. We have demonstrated that “normal-appearing” scalp that is devoid of clinical lesions of LPP and FFA showed lymphocytic perifollicular inflammation around the isthmus/infundibulum areas in 65% of biopsy specimens, perifollicular fibrosis in 15% and mucin deposits in 7.5% of the cases. None of these findings were found in control samples. No direct correlation was found between the degree of histopathological inflammation, scalp symptoms and clinical lesions in the corresponding affected scalp areas. This preliminary study suggests that both diseases may be more generalized processes which affect the scalp and therefore need systemic or total scalp therapy.     

雄秃样纤维性秃发一例

【摘要】 报道1例雄秃样纤维性秃发（FAPD）及其临床病理、皮肤镜和TrichoScan特征，以提高对FAPD的认识。患者男，23岁，前额及头顶部进行性脱发10年，伴局部头发变细、软，偶有头皮瘙痒。皮肤科检查：前额至头顶部头发弥漫性稀疏，前额发际线后移，局部发质细软，脱发区可见部分毛囊角化性丘疹、毛囊周围红斑，未见明显鳞屑。TrichoScan检查：毛发密度明显降低，毳毛比例增加。皮肤镜检查：部分毛囊开口消失，融合性白点征。头皮组织病理检查：毛囊漏斗部、峡部淋巴细胞苔藓样浸润，毛囊周围同心层状纤维化，毛囊结构破坏，毛囊性微瘢痕形成，残留毛囊直径变异度明显增加，可见部分毳毛。诊断：FAPD。FAPD临床易误诊为雄激素性秃发，需及早诊断并治疗。     

Frontal fibrosing alopecia

A 75-year-old woman presented with a 3-year history of progressive loss of her eyebrow hair and with frontal-parietal hairline recession. Multiple biopsy specimens supported a histopathologic diagnosis of lichen planopilaris. With these histolopathologic findings, and the patient's clinical presentation, a diagnosis of frontal fibrosing alopecia was made. Treatment to date with topical glucocorticoid preparations, intralesional triamcinolone injections, and tacrolimus ointment have been unsuccessful.     

Combination antimalarials in the treatment of cutaneous dermatomyositis: a retrospective study

OBJECTIVE: To observe whether the use of antimalarials in combination resulted in significant improvement in the cutaneous signs and symptoms of patients with dermatomyositis who did not otherwise respond to the use of single-agent antimalarial therapy. DESIGN: Retrospective case series of 17 patients treated between January 1, 1991, and December 31, 2002. SETTING: An ambulatory medical dermatology clinic in an academic center.Patients Patients had adult-onset dermatomyositis with predominantly cutaneous symptoms and a follow-up period at our clinic of at least 6 months. Cases in which it was not possible to assess the effect of treatment on cutaneous symptoms were not included.Intervention Treatment regimens varied and included the use of antimalarials, prednisone, methotrexate, and other medications. MAIN OUTCOME MEASURES: Physician-observed and patient-reported improvement based on erythema, pruritus, and extent of affected skin. RESULTS: Seven of 17 patients experienced at least near clearance in cutaneous symptoms with the use of antimalarial therapy alone: 4 of these patients required combination therapy (hydroxychloroquine sulfate-quinacrine hydrochloride or chloroquine phosphate-quinacrine), while 3 of them responded well to antimalarial monotherapy. The median time required to reach the response milestones on the final working therapeutic regimen was 3 months (mean, 4.8 months; range, 2-14 months). Six patients did not respond significantly to any type of therapy, including nonantimalarials. CONCLUSION: Our experience suggests that a significant subgroup of patients whose skin lesions have been unresponsive to a single antimalarial benefit from combination therapy with hydroxychloroquine and quinacrine or chloroquine and quinacrine, but controlled clinical trials are warranted to assess the extent of benefit.     

Using biopsies to study the differences between two hair loss disorders: lichen planopilaris and frontal fibrosing alopecia

Lichen planopilaris (LPP) and frontal fibrosing alopecia (FFA) are two uncommon disorders that both cause permanent, scarring alopecia (hair loss) and inflammation of the hair follicles. LPP affects both sexes and typically causes patchy alopecia over the central scalp, whereas FFA generally affects women and causes the hairline at the front of the scalp to recede. It can also cause eyebrow and body hair loss. Despite these distinct phenotypes (physical characteristics), FFA has traditionally been regarded as a variant of LPP. The authors sought to distinguish the two conditions using a wide array of markers for different cell types around the hair follicles. Markers are substances in the body, such as certain proteins, which act as signs to show, for example, the presence of certain cells or inflammation. The authors took skin punch biopsies (a type of tissue sample) from affected areas of scalp in patients with a diagnosis of LPP or FFA, and from healthy controls (people without LPP or FFA). In this study, the authors present a detailed, systematic analysis of a range of key inflammatory cell markers in LPP, FFA and controls. There were many similarities in the numbers and type of inflammatory cells in the two conditions. The study focussed on the different types of cells called macrophages seen in LPP and FFA. Broadly, these cells can be divided into two types, M1 cells, associated with inflammation and cell death, and M2 cells, involved in tissue repair. The two conditions differed in that more markers for M2 cells were seen in LPP in comparison to FFA. The authors hope that therapies directed against specific macrophage cell types may be developed in the future, preventing irreversible hair loss. Linked Article:   Harries et al. Br J Dermatol 2020; 183 :537–547 .     

Histopathology of keratotic papules of the limbs in frontal fibrosing alopecia

Frontal fibrosing alopecia (FFA) is a scarring type of alopecia that presents clinically as progressive frontotemporal hairline regression and eyebrow loss, mainly in postmenopausal women. An additional common finding is keratotic papules on the face or (less commonly) on the trunk and extremities. The histopathology of the facial keratotic papules associated with FFA is the same as that of lichen planopilaris. There are very few FFA cases with biopsies from extrafacial sites and no cases of folliculocentric keratotic papules with biopsies from extrafacial sites. In the current report, we describe the histopathology of one such keratotic papule from the arm of a 75‐year‐old woman with FFA and show that the morphology is that of lichen planopilaris and that the papules are identical to those found in Graham–Little–Piccardi syndrome.