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Ralph M. Trüeb - One of the best experts on this subject based on the ideXlab platform.
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hyperpigmented upper eyelid a clue to the diagnosis of facial lichen planus pigmentosus in a patient with Frontal Fibrosing Alopecia
Skin Appendage Disorders, 2018Co-Authors: Maria Fernanda Reis Gavazzoni Dias, Ralph M. Trüeb, Hudson Dutra Rezende, Amanda Lofeu Cury, Enoi Aparecida Guedes VilarAbstract:Facial lichen planus pigmentosus (LPPig), a rare variant of classic lichen planus, was first described in patients with Frontal Fibrosing Alopecia (FFA) by Dlova [Br J Dermatol 2013; 168: 439-442] in 2013. The diagnosis of facial LPPig is sometimes not easy, since clinical signs and histopathological features may frequently be confused with melasma or postinflammatory hyperpigmentation. We describe a case of a postmenopausal black woman diagnosed with FFA who presented with an identical brown-grayish pigmentation of the face and upper eyelids and typical dermoscopy analysis on both regions. We suggest that the hyperpigmentation of the upper eyelid with typical LLPig dermoscopy (upper eyelid sign) may be a clue for the diagnosis of LPPig and may avoid a scar-causing face biopsy.
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Familial Cicatricial Alopecia: Report of Familial Frontal Fibrosing Alopecia and Fibrosing Alopecia in a Pattern Distribution.
International Journal of Trichology, 2017Co-Authors: Dandara Meurer Missio, Maria Fernanda Reis Gavazzoni Dias, Ralph M. TrüebAbstract:Frontal Fibrosing Alopecia (FFA) and Fibrosing Alopecia in a pattern distribution (FAPD) as originally reported by Kossard in 1994 and by Zinkernagel and Trueb in 2000, respectively, represent two distinct patterns of cicatricial pattern hair loss. Both share a patterned distribution and histological evidence of a lichenoid follicular inflammation with fibrosis. FFA is characterized by a marginal Alopecia along the frontotemporal hairline, and FAPD by a progressive Alopecia of the centroparietal scalp. Since the original reports, evidence has accumulated that there exists considerable clinical overlap among FFA, FAPD, and lichen planopilaris, with coexistence of features of the three conditions within the same individual. Moreover, familial cases of FFA have been reported, pointing to a possible genetic background to the condition. Our observation of familial occurrence of FFA and FAPD in daughter and mother, respectively, further underscore a nosologic relationship between the two conditions with respect to both an androgenetic background and the (lichenoid) inflammatory reaction pattern.
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facial papules in Fibrosing Alopecia in a pattern distribution cicatricial pattern hair loss
International Journal of Trichology, 2015Co-Authors: Ausrine Ramanauskaite, Ralph M. TrüebAbstract:Frontal Fibrosing Alopecia (FFA) and Fibrosing Alopecia in a pattern distribution (FAPD) represent clinically distinctive conditions characterized by pattern hair loss with evidence of follicular inflammation and fibrosis. Since Kossard's original description, the condition has been recognized to represent a rather generalized than localized process, with extension well beyond the frontotemporal hairline. More recently, peculiar facial papules have been reported in FFA representing facial vellus hair involvement. We report the case of a 42-year-old woman with FAPD associated with the same facial papules, supporting that both entities belong to the same spectrum of cicatricial pattern hair loss.
Antonella Tosti - One of the best experts on this subject based on the ideXlab platform.
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pili torti as a sign of eyebrow involvement in Frontal Fibrosing Alopecia
Skin Appendage Disorders, 2019Co-Authors: Bruno Ferrari, Colombina Vincenzi, Antonella TostiAbstract:Frontal Fibrosing Alopecia (FFA) is a disease characterized by progressive band-like scarring Alopecia involving the frontotemporal hairline and eyebrow hair loss. It affects mainly postmenopausal women. Trichoscopy features of FFA include absence of vellus hair, perifollicular erythema and scaling (peri-pilar casts), and absence of follicular openings. Trichoscopy of eyebrows in FFA patients shows tapered and broken hair, absence of follicular openings, black dots, and hair growing in different directions. We report a case of FFA with numerous pili torti in the eyebrows.
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medical comorbidities in patients with lichen planopilaris a retrospective case control study
International Journal of Dermatology, 2018Co-Authors: Raymond M Fertig, Shasa Hu, Austin J Maddy, Alexandra Balaban, Nouf M Aleid, Adam S Aldahan, Antonella TostiAbstract:BACKGROUND: Lichen planopilaris (LPP) is a rare inflammatory lymphocyte-mediated disease of the scalp considered to have an autoimmune pathogenesis. OBJECTIVES: To identify the prevalence of medical comorbidities in patients with classic LPP (CLPP) and Frontal Fibrosing Alopecia (FFA). METHODS: The medical records of 206 LPP patients and 323 control patients were retrospectively reviewed for existing comorbidities. The control group consisted of 257 patients with androgenetic Alopecia (ICD 9 = 704.0 or ICD 10 = L64.9) and 66 patients with actinic keratosis (ICD 9 = 702.0 or ICD 10 = L57.0). RESULTS: Systemic lupus erythematosus (SLE) was found in 4.37% of all patients with LPP (including CLPP and the FFA subtype) and in 0.31% of controls. Female patients with the FFA subtype were more likely to have SLE than controls (OR 31.034, 95% CI 2.405-400.382, P = 0.0085). LIMITATIONS: This study is limited in that it is a retrospective chart review. CONCLUSION: Female patients with FFA are significantly more likely to have SLE. Patients with LPP (including CLPP and the FFA subtype) are less likely to have diabetes. Patients with CLPP excluding FFA are less likely to have hypertension, heart disease, and hypothyroidism.
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Frontal Fibrosing Alopecia severity index a trichoscopic visual scale that correlates thickness of peripilar casts with severity of inflammatory changes at pathology
Skin Appendage Disorders, 2018Co-Authors: Maria Abril Martinezvelasco, Austin J Maddy, Colombina Vincenzi, Cosimo Misciali, Norma Elizabeth Vazquezherrera, Daniel Aszsigall, Antonella TostiAbstract:Background Frontal Fibrosing Alopecia (FFA) is a scarring Alopecia that mainly affects postmenopausal women characterized by recession of the frontotemporal hairline and eyebrow loss. Current techniques to assess FFA activity are limited and involve noninvasive tools that assess disease progression or an invasive technique such as scalp biopsies. However, since progression of FFA is very slow, it is very important to develop a noninvasive technique to assess disease activity to monitor treatment response. Objectives To provide a standardized and objective method to assess FFA activity. Methods We evaluated the correlation between trichoscopy and pathological features (degree of lymphocytic infiltration) in 20 dermoscopy-guided biopsies of FFA. At trichoscopy, we divided the severity of peripilar casts into 3 grades according to their thickness. To validate the trichoscopic visual scale, we showed the images to 7 dermatologists with interest in hair diseases. Concordance was assessed using the Kendall Tau-b concordance test. Results A strong correlation between severity of peripilar casts at trichoscopy and degree of lymphocytic infiltrate was observed by the Kendall Tau-b test. Validation showed very good inter- and intraobserver agreement. Conclusion The trichoscopic visual scale allows noninvasive assessment of scalp inflammation in FFA in different scalp regions and therefore provides optimal guidance for treatment.
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open access to scientific and medical research Review
2013Co-Authors: Katherine A. Gordon, Antonella TostiAbstract:Abstract: Hair loss is a very common complaint. Patients may describe increased shedding and diffuse or localized Alopecia. The differential diagnosis of hair loss includes a number of disorders causing cicatricial or noncicatricial Alopecias. This paper describes the clinical approaches and diagnostic tests that are useful in the evaluation of patients presenting with Alopecia. It also reviews treatments for noncicatricial Alopecias, including androgenetic Alopecia, Alopecia areata, and telogen effluvium, as well as cicatricial Alopecias, including lichen planopilaris, its clinical variant Frontal Fibrosing Alopecia, and discoid lupus erythematosus
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dermoscopy guided scalp biopsy in cicatricial Alopecia
Journal of The European Academy of Dermatology and Venereology, 2012Co-Authors: Mariya Miteva, Antonella TostiAbstract:Background Scalp biopsies are crucial for the diagnosis of cicatricial Alopecia. However, the pathologic interpretation may not be diagnostic if biopsy is not obtained from the correct site. This is particularly relevant for cicatricial Alopecia as the disease may be focal and disease activity difficult to appreciate by the naked eye. Objective To report a new simple technique to select the optimal biopsy site in cicatricial Alopecia. Methods In the last 2 years we performed dermoscopy guided scalp biopsies using handled dermatoscopes in 80 patients with different forms of cicatricial Alopecia. Biopsy site was selected based on presence of the following dermatoscopic features: perifollicular concentric white scales in lichen planopilaris, Frontal Fibrosing Alopecia (FFA) and discoid lupus erythematosus (DLE); hair tufts in folliculitis decalvans, hairs surrounded by a peripilar grey-white halo in central centrifugal cicatricial Alopecia and follicular red dots or keratotic plugs in DLE. Results The dermoscopy guided biopsies yielded a definitive pathological diagnosis in 95% of the cases. Comment The advantage of this method is that it is a fast, precise way to identify even individually affected follicles in early or focal cicatricial Alopecia. It also allows for the morphologic characterization of particular follicular structures.
Steven Kossard - One of the best experts on this subject based on the ideXlab platform.
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postmenopausal Frontal Fibrosing Alopecia a Frontal variant of lichen planopilaris
Journal of The American Academy of Dermatology, 1997Co-Authors: Steven Kossard, Barbara WilkinsonAbstract:Abstract Background: Lichen planopilaris usually produces multifocal areas of scarring Alopecia. Recently, a condition in postmenopausal women characterized by progressive Frontal hairline recession associated with scarring has been described. Objective: Our purpose was to study the clinical and histopathologic features and results of treatment in a group of women with the Frontal variant of lichen planopilaris and to compare the immunohistochemical profile of scalp biopsy specimens from this subset with that found in the multifocal variant of lichen planopilaris. Method: The clinical data as well as the histopathologic findings in 16 women with Frontal Fibrosing Alopecia were collated. The immunohistochemical profile of six scalp biopsy specimens from the Frontal hairline were compared with six specimens from women with multifocal lichen planopilaris. Results: In addition to the progressive Frontal Fibrosing Alopecia in all 16 women, total loss or a marked decrease of the eyebrows was observed in 13. No evidence of lichen planus was observed at other sites. In one patient multifocal areas of lichen planopilaris developed in the scalp. The Frontal Fibrosing Alopecia was slowly progressive but has stabilized in five patients. Biopsy specimens from the Frontal hairline showed histologic changes identical to lichen planopilaris. Immunophenotyping failed to reveal any significant differences between the Frontal and multifocal variants. No effective treatments emerged although oral steroids and antimalarials may temporarily slow the course. Hormone replacement therapy did not appear to influence the course of the Alopecia. Conclusion: Progressive Frontal Fibrosing Alopecia is a clinically distinct variant of lichen planopilaris that affects in particular elderly women and frequently involves the eyebrows. The basis for this lichenoid tissue reaction targeting Frontal scalp follicles and eyebrows is unknown. (J Am Acad Dermatol 1997;36:59-66.)
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postmenopausal Frontal Fibrosing Alopecia scarring Alopecia in a pattern distribution
Archives of Dermatology, 1994Co-Authors: Steven KossardAbstract:Background: Recession of the Frontal hairline is a common event in postmenopausal women. This has been shown not to be a marker of gross androgenization, and is usually a progressive nonscarring Alopecia. Six postmenopausal women, who developed a progressive Frontal scarring Alopecia, were studied and their clinical and laboratory data, as well as the results of scalp biopsy specimens in all six patients, were analyzed and compared with recognized forms of scarring Alopecia and recently described findings in androgenetic Alopecia. Observations: The six postmenopausal women developed a progressive Frontal hairline recession that was associated with perifollicular erythema within the marginal hairline, producing a Frontal Fibrosing Alopecia extending to the temporal and parietal hair margins. Scalp biopsy specimens from the Frontal hair margin showed perifollicular fibrosis and lymphocytic inflammation concentrated around the isthmus and infundibular areas of the follicles. Immunophenotyping of the lymphocytes showed a dominance of activated T-helper cells. Clinical review of all six cases showed a progressive marginal Alopecia without the typical multifocal areas of involvement seen in lichen planopilaris or pseudopelade. None of the patients had mucous membrane or skin lesions typical of lichen planus. Hormonal studies, in five patients, showed no elevated androgen abnormalities. Conclusions: Progressive Frontal recession in postmenopausal women may show clinical features of a Fibrosing Alopecia. The histologic findings are indistinguishable from those seen in lichen planopilaris. However, the absence of associated lesions of lichen planus in all six women raises the possibility that this mode of follicular destruction represents a reaction pattern triggered by the events underlying postmenopausal Frontal hairline recession. (Arch Dermatol. 1994;130:770-774)
M Harries - One of the best experts on this subject based on the ideXlab platform.
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Frontal Fibrosing Alopecia possible association with leave on facial skin care products and sunscreens a questionnaire study
British Journal of Dermatology, 2016Co-Authors: N Aldoori, K Dobson, Catherine R Holden, M Harries, Andrew J. G. Mcdonagh, Andrew G. MessengerAbstract:SummaryBackground Since its first description in 1994, Frontal Fibrosing Alopecia (FFA) has become increasingly common, suggesting that environmental factors are involved in the aetiology. Objectives To identify possible causative environmental factors in FFA. Methods A questionnaire enquiring about exposure to a wide range of lifestyle, social and medical factors was completed by 105 women with FFA and 100 age- and sex-matched control subjects. A subcohort of women with FFA was patch tested to an extended British standard series of allergens. Results The use of sunscreens was significantly greater in the FFA group compared with controls. Subjects with FFA also showed a trend towards more frequent use of facial moisturizers and foundations but, compared with controls, the difference in frequencies just failed to reach statistical significance. The frequency of hair shampooing, oral contraceptive use, hair colouring and facial hair removal were significantly lower in the FFA group than in controls. Thyroid disease was more common in subjects with FFA than controls and there was a high frequency of positive patch tests in women with FFA, mainly to fragrances. Conclusions Our findings suggest an association between FFA and the use of facial skin care products. The high frequency of sunscreen use in patients with FFA, and the fact that many facial skin care products now contain sunscreens, raises the possibility of a causative role for sunscreen chemicals. The high frequency of positive patch tests in women with FFA and the association with thyroid disease may indicate a predisposition to immune-mediated disease.
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Frontal Fibrosing Alopecia severity index ffasi a validated scoring system for assessing Frontal Fibrosing Alopecia
British Journal of Dermatology, 2016Co-Authors: S Holmes, T Ryan, David Young, M HarriesAbstract:The incidence of Frontal Fibrosing Alopecia (FFA) appears to be increasing(1-6) and response to treatment has been largely disappointing(1-3) . However, assessment of treatment interventions is confounded by slow disease progression and lack of robust means of assessing disease severity and activity. To address the latter, we have developed a validated clinical scoring system - the FFA severity index (FFASI), which provides a standardised framework for FFA assessment and patient.
Jerry Shapiro - One of the best experts on this subject based on the ideXlab platform.
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primary cicatricial Alopecia
Journal of The American Academy of Dermatology, 2016Co-Authors: Chantal Bolduc, Jerry Shapiro, Leonard C. SperlingAbstract:Both primary and secondary forms of cicatricial Alopecia have been described. The hair follicles are the specific target of inflammation in primary cicatricial Alopecias. Hair follicles are destroyed randomly with surrounding structures in secondary cicatricial Alopecia. This 2-part continuing medical education article will review primary cicatricial Alopecias according to the working classification suggested by the North American Hair Research Society. In this classification, the different entities are classified into 3 different groups according to their prominent inflammatory infiltrate (ie, lymphocytic, neutrophilic, and mixed). Part I discusses the following lymphocytic primary cicatricial Alopecias: chronic cutaneous lupus erythematosus, lichen planopilaris, Frontal Fibrosing Alopecia, and Graham–Little syndrome.
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Frontal Fibrosing Alopecia a retrospective clinical review of 62 patients with treatment outcome and long term follow up
International Journal of Dermatology, 2014Co-Authors: Nusrat Banka, Thamer Mubki, Marry Jo Kristine Bunagan, Kevin J Mcelwee, Jerry ShapiroAbstract:Background Frontal Fibrosing Alopecia is a distinctive form of scarring Alopecia presenting with Frontal and temporoparietal recession of the hairline. Its etiology remains unknown, and there are no universal treatment guidelines. We conducted a retrospective cohort study to define the clinical findings and treatment outcomes of 62 patients with Frontal Fibrosing Alopecia, one of the largest cohorts to date. Methods Data analysis from case notes was performed on 62 patients with a diagnosis of Frontal Fibrosing Alopecia seen from January 2004 to March 2012. Results Except for one male, all patients in this cohort were females (80% post-menopausal) and mostly Caucasians (81%). Age at onset was between 18 and 81 years. While 35% reported no symptoms, the majority (65%) had itching, pain, or burning sensations. All patients had Frontal hairline recession, and 81% had complete or partial loss of eyebrows. Perifollicular erythema and perifollicular hyperkeratosis occurred in 73% and 31%, respectively. Associated autoimmune connective tissue diseases were observed in 14% of patients. Reduction in symptoms and hairline stabilization were achieved in 97% of treated patients with intralesional corticosteroids. Thirty-one percent of patients were able to stop treatments and remained in remission for six months to six years. Conclusion Frontal Fibrosing Alopecia is increasingly seen in postmenopausal women and rarely in men. Despite the limitations of a retrospective study, we conclude early intervention and treatment with intralesional triamcinolone acetonide may halt the progression of the disease; however, further controlled prospective studies are needed to establish treatment guidelines for Frontal Fibrosing Alopecia.
