RESUMO
Postmenopausal lichen planopilaris (PLPP), also known as fibrosing frontotemporal alopecia Kossard (FFAK), is a not uncommon inflammatory scalp disease affecting approximately 5% of patients at specialized hair centers. The overall incidence of sporadic occurrence is believed to be just under 1% in the older, predominantly female, general population. Since the disease is often undiagnosed, it is statistically likely to be underrepresented. It especially occurs in postmenopausal women who are in the 6th and 7th decade of life (90%), but also in about 10% of premenopausal women, and in men it is documented only in isolated cases. The result is a permanent scarring hair loss accentuated at the front hairline with backward movement towards the neck mostly accompanied by a typical loss of the eyebrows. The disease therefore often leads to significant mental distress and social anxiety in those affected. This is the basis for a compelling need to develop evidence-based therapeutic concepts. While numerous retrospective case series have characterized the phenomenology of FFAK very well, to date there are no randomized controlled trials on evidence-based therapy. Here, we present the Homburger Evidence-Oriented Therapy Algorithm, which is oriented along the available case series evidence: It may (1) serve as a therapy guide for practice and (2) can be used as a basis for working out reliable data based on study evidence. The article contains detailed practical information on photo documentation, biopsy and histological processing up to the practical implementation of, for example, intralesional steroid therapy as well as information on selection criteria for suitable systemic therapies.
Assuntos
Alopecia/diagnóstico , Líquen Plano/diagnóstico , Pós-Menopausa , Adulto , Idoso , Algoritmos , Alopecia/patologia , Alopecia/terapia , Diagnóstico Diferencial , Medicina Baseada em Evidências , Feminino , Fibrose , Finasterida/uso terapêutico , Humanos , Inibidores de Janus Quinases/uso terapêutico , Líquen Plano/patologia , Líquen Plano/terapia , Masculino , Pessoa de Meia-Idade , Couro Cabeludo/patologiaRESUMO
Frontal fibrosing alopecia (FFA) was first described in 1994. It is characterized by scarring alopecia in bands involving the anterior area of the scalp. Alopecia of the eyebrows is frequently associated, as are pubic, facial and body hair alopecia. The clinical and histologic features are evocative of lichen planopilaris (LPP), and AFF is in fact regarded as a special pattern of LPP. Histology reveals a lymphocytic infiltrate located around the isthmus and follicular infundibulum associated with a decrease in the number of follicles, which are supplanted by fibrous tract. AFF most commonly affects post-menopausal women, but instances have been described in men and in young women. This orphan disease has increased in recent years, with more than 37 articles dedicated to this condition since it was first described in 1994. The pathophysiology remains unknown. The condition develops slowly with spontaneous stabilization over several years but it is impossible to predict the degree of expression prior to stabilization. In this article we review the various treatments proposed, for none of which formal proof of efficacy has been provided to date.