Fenómeno infrecuente la co-localización de alopecia frontal fibrosante y vitiligo en un varón: informe de un caso / Infrequent phenomenon the co-localization of fibrosing frontal alopecia and vitiligo in a male: case repor

La alopecia frontal fibrosante es una alopecia cicatricial que se caracteriza por la recesión de la línea de implantación frontotemporal que afecta principalmente a mujeres caucásicas en edad posmenopáusica y rara vez a hombres. Actualmente los mecanismos específicos de desarrollo continúan en estudio; sin embargo hay varias hipótesis sobre la asociación de la alopecia frontal fibrosante con otros trastornos autoinmunitarios. Se comunica el caso de un paciente masculino de 58 años con alopecia frontal fibrosante en áreas comprometidas por vitiligo. (AU)

Frontal fibrosing alopecia is a cicatricial alopecic characterized by progressive regression of the frontotemporal hairline. It usually affects postmenopausal caucasian women, and rarely men. Currently the specific mechanisms of development remain unknown, however there are several hypotheses about the association of frontal fibrosing alopecia with other autoimmune disorders. The case of a 58-year-old male patient with frontal fibrosing alopecia in areas affected by vitiligo. (AU)

Frontal fibrosing alopecia: two case reports / 中华皮肤科杂志

Two cases of frontal fibrosing alopecia (FFA) were reported.Case 1,a 44-year-old woman,presented with progressive recession of the frontotemporal hairline for 4 years and multiple skincolored facial papules for 2 years.Skin examination showed that the frontotemporal hairline was receded,the local skin was smooth and thin,and a few remaining fine hairs could be seen.Additionally,eyebrows,axillary and pubic hairs were partly shed,and plenty of millet-sized skin-colored papules were diffusedly distributed on the frontotemporal region and bilateral mandibular angles.Dermatoscopy showed reduced hair follicular openings,different hair shaft diameters,cicatricial white patches and perifollicular erythema.Case 2,a 55-year-old woman,presented with progressive frontotemporal hair loss for 2 years.Skin examination revealed the recession of bilateral frontotemporal hairline and partial loss of eyebrows,axillary and pubic hairs.Histopathological examinations of the 2 patients both revealed perifollicular infiltration of lymphocytes,liquefaction degeneration of basal cells and perifollicular lamellar fibrosis.Clinical manifestations and histopathological features of the 2 patients both confirmed the diagnosis of FFA.

DHEA and frontal fibrosing alopecia: molecular and physiopathological mechanisms

Abstract The transforming growth factor-beta 1 (TGFβ1) promotes fibrosis, differentiating epithelial cells and quiescent fibroblasts into myofibroblasts and increasing expression of extracellular matrix. Recent investigations have shown that PPAR (peroxisome proliferator-activated receptor*) is a negative regulator of fibrotic events induced by TGFβ1. Dehydroepiandrosterone (DHEA) is an immunomodulatory hormone essential for PPAR functions, and is reduced in some processes characterized by fibrosis. Although scarring alopecia characteristically develops in the female biological period in which occurs decreased production of DHEA, there are no data in the literature relating its reduction to fibrogenic process of this condition. This article aims to review the fibrogenic activity of TGFβ1, its control by PPAR and its relation with DHEA in the frontal fibrosing alopecia.

Frontal fibrosing alopecia and lichen planopilaris in HLA-identical mother and daughter.

Frontal fi brosing alopecia (FFA) is a lymphocyte-mediated scarring alopecia thought to be a variant of lichen planopilaris (LPP). We present a 67-year-old woman with frontal fi brosing alopecia whose daughter was diagnosed to have lichen planopilaris. Both patients had identical human leukocyte antigen (HLA) D types, supporting a phenotypical relationship between the two clinical entities. Interestingly, our patient also had of autoimmune chronic atrophic gastritis, a previously unreported association.

Estudio retrospectivo de pacientes con alopecia fibrosante frontal / Retrospective study of patients with frontal fibrosing alopecia

Introducción: La alopecia fibrosante frontal, considerada por algunos autores como un subtipo de liquen plano pilaris, corresponde a una alopecia cicatricial primaria, que se presenta en mujeres adultas y se caracteriza por el retroceso simétrico de la línea de implantación fronto temporal. Se presenta un estudio descriptivo de pacientes con Alopecía fibrosante frontal atendidos en este centro. Objetivos: Evaluar características clínicas, dermatoscópicas e histopatológicas de pacientes con alopecia fibrosante frontal. Materiales y métodos: Estudio retrospectivo de pacientes con diagnóstico clínico-histopatológico de alopecia fibrosante frontal atendidos en nuestro centro desde Julio 2010 a Octubre 2012. Resultados: Se encontraron 57 casos con diagnóstico histológico de alopecia cicatricial, de los cuales 8 (14 por ciento) se diagnosticaron como Alopecia Fibrosante Frontal. El 100 por ciento correspondieron a mujeres, el promedio de edad fue de 45 años. Sólo la mitad de los casos comenzó con la alopecia luego del climaterio. El hallazgo clínico más frecuentemente encontrado fue el retroceso de la línea de implantación frontotemporal (87,5 por ciento); y en segundo lugar la disminución difusa de densidad capilar (37,5 por ciento) y alopecia de la cola de las cejas (50 por ciento). No se reportó ningún caso asociado a liquen plano cutáneo o de mucosas. Los hallazgos más relevantes a la dermatoscopía fueron: eritema perifolicular (50 por ciento) e hiperqueratosis folicular (25 por ciento). A la histopatología, los principales hallazgos fueron el infiltrado inflamatorio linfocitario perifolicular (50 por ciento) y fibrosis concéntrica perifolicular (100 por ciento). Discusión: La Alopecia fibrosante frontalrepresenta el 14 por ciento de las alopecias cicatriciales primarias en nuestra serie. La edad al diagnóstico fue inferior a lo reportado en la literatura. El principal hallazgo clínico fue el retroceso de la línea de implantación frontotemporal...

Introduction: Frontal fibrosing alopecia, considered by some authors as a subtype of lichen planopilaris, is a scarring alopecia that usually involves adult women and is characterized by the symmetric recession of fronto-temporal hairline. Objectives: Characterize the clinical, dermoscopic and histological features of frontal fibrosing alopecia. Materials and Methods: We conducted a retrospective study of patients with clinical and histological diagnosis of Frontal Fibrosing Alopecia treated in our institution from July 2010 to October2012. Results: 8 out of 57 cases with histological diagnosis of scarring alopecia, had diagnosis of frontal fibrosing alopecia (14 percent). 100 percent were women, with mean age of 45 years. 50 percent of patients began with alopecia in the post-climacteric period. Recession of frontotemporal hair-line was the most common finding (87.5 percent), associated with reduced capillary density (37.5 percent) and loss of eyebrows (50 percent). In our study, none of the patients had other signs of lichen planus. At dermoscopy, most common finding were perifollicular erythema (50 percent) and follicular hyperkeratosis (25 percent). Most common histological findings were a perifollicular lymphocytic inflammatory infiltrate (50 percent) and perifollicular concentric fibrosis (100 percent). Discussion: Frontal fibrosing alopecia represents 14 percent of scarring alopecia in our series. Age at diagnosis was lower than reported in the literature, and only 50 percent of women presented alopecia in the postmenopausal period. The main clinical finding was the recession of frontotemporal hairline...

Frontal Fibrosing Alopecia with Partial Improvement after Systemic Steroid Administration and Topical Calcineur in Inhibitor Application / 대한피부과학회지

Frontal fibrosing alopecia (FFA) is a rare subtype of cicatricial alopecia. It was first described in 1994 by Kossard as postmenopausal frontal fibrosing alopecia. Patients who suffer from FFA show typical frontal hairline recession, and most patients experience eyebrow loss. It usually affects mainly post-menopausal women but the hormonal change due to menopause is unclear. Etiology of FFA is not clear, but it is assumed that certain autoantigens in the frontal and eyebrow hair follicles play a key role in its pathogenesis. There is no optimal treatment thus far. However, recently, topical calcineurin inhibitor was shown to be effective in early stage FFA via follicular targeted T-cell inhibition. Here, we report a case of a premenopausal 50-year-old female patient suffering from FFA displaying typical clinical features and minimal fibrosis around follicles by histological examination, which were improved by treatment with short-term systemic steroid and long-term topical calcineurin inhibitor.

Clinicopathological Observation of Frontal Fibrosing Alopecia / 대한피부과학회지

BACKGROUND: Frontal fibrosing alopecia (FFA), typically observed in elderly women, is characterized by a band of frontal or frontoparietal hair recession with scarring and a marked decrease, or a complete loss of the eyebrows. OBJECTIVE: To describe the clinical and histopathological features, and response to treatment of FFA in Korean patients. METHODS: A total of 7 female patients, diagnosed as FFA at our department from 2003 through 2006, were evaluated. RESULTS: All patients presented with a band of symmetric recession of the frontoparietal hairline. Four patients (57.1%) had symmetric loss of lateral eyebrows. Two patients (28.6%) were premenopausal. The histologic features were similar in all patients with a reduction of the number of hair follicles, and a perifollicular lymphocytic infiltration with lamellar fibrosis limited to the upper portions of the follicles. The progression of the condition stopped in all patients after topical steroid treatment. CONCLUSION: Cases of FFA affected in premenopausal female patients drove us to consider that this condition is not exclusive to postmenopausal females. To make a diagnosis of FFA, we should remind ourselves of the typical clinical and pathologic features. Further studies are necessary to confirm the possible role of treatment in FFA and stop the progression in the early stages of FFA.

A Case of Frontal Fibrosing Alopecia / 대한피부과학회지

Frontal fibrosing alopecia (FFA) is one of the primary cicatricial alopecia and is considered a variant of lichen planopilaris. This condition is characterized by a progressive recession of the frontal and temporal hairline, primarily in postmenopausal women. Only two cases have been reported in premenopausal woman worldwide. We report a case of FFA in a 45-year-old female in her premenopausal age, who presented with multiple, small, bald spots on the bilateral frontotemporal area. Microscopically the lesion consisted of the inflammatory stage of lichen planopilaris and its cicatricial stage showing replacement of hair follicles by fibrous tissue. This was an additional case of frontal fibrosing alopecia in a premenopausal woman.

A Case of Frontal Fibrosing Alopecia / 대한피부과학회지

Frontal fibrosing alopecia is considered to be a clinical variant of lichen planopilaris. This condition is characterized by progressive frontal hairline recession associated with scarring. Frontal fibrosing alopecia has become an increasingly recognized distinct clinical entity in recent years. We report a typical case of frontal fibrosing alopecia and review the pertinent literature.

A case of postmenopausal frontal fibrosing alopecia / 대한피부과학회지

Postmenopausal frontal fibrosing alopecia is a recently described, rare scarring alopecia. The disease is characterized by progressive frontal recession in postmenopausal women with clinical features of a fibrosing alpecia. The histologic findings are indistinguishable from those seen in lichen planopilaris. Postmenopausal frontal fibrosing alopecia is especially rare in Asian women. We describe a typical case of postmenopausal frontal fibrosing alpecia in a Korean woman with clinical and histologic features.