Zusammenfassung
Der postmenopausale Lichen planopilaris (PLPP) alias fibrosierende frontotemporale Alopezie Kossard (FFAK) ist eine nicht seltene entzündliche Erkrankung der Kopfhaut und betrifft etwa 5 % der Patienten an spezialisierten Haarzentren. Die allgemeine Inzidenz des sporadischen Vorkommens liegt vermutlich bei knapp 1 % in der älteren, vorrangig weiblichen Allgemeinbevölkerung. Da die Erkrankung oft nicht diagnostiziert wird, ist sie statistisch wahrscheinlich unterrepräsentiert. Sie kommt speziell bei Frauen betont in der 6. und 7. Lebensdekade postmenopausal vor (um 90 %), etwa in 10 % aber auch prämenopausal. Bei Männern ist sie nur in Einzelfällen dokumentiert. Die Folge ist ein bleibender vernarbender Haarausfall, betont am vorderen Haaransatz mit Rückwärtsbewegung des Ansatzes, begleitet von einem regelhaften, typischen Verlust auch der Augenbrauen. Die Erkrankung führt daher bei Betroffenen oft zu erheblichen psychischen Belastungen und sozialen Ängsten. Hieraus leitet sich das zwingende Erfordernis ab, Therapiekonzepte evidenzbasiert zu entwickeln. Während zahlreiche retrospektive Fallserien die Phänomenologie der FFAK sehr gut charakterisiert haben, fehlen bis heute randomisierte kontrollierte Studien zur evidenzbasierten Therapie. Wir stellen hier den Homburger evidenzorientierten Therapiealgorithmus vor, der so weit an Fallserienevidenzen orientiert ist, dass er a) als Therapieanleitung für die Praxis dienen und b) als Arbeitsgrundlage herangezogen werden kann, um zukünftig belastbare, auf Studienevidenz basierte Daten zu erarbeiten. Der Beitrag enthält detaillierte praktische Hinweise zur Fotodokumentation, zur Biopsieentnahme und histologischen Aufarbeitung bis hin zur praktischen Durchführung etwa der intraläsionalen Steroidtherapie sowie Hinweise zu Auswahlkriterien geeigneter Systemtherapien.
Abstract
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.
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Danksagung
Wir danken der Dr. Rolf M. Schwiete Stiftung für die Unterstützung der Haarforschung am UKS.
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T. Vogt, C. Thomas, J. Reichrath, L. Schilling, D. Mawlood, R. Christmann, B. Loretz, U. Schäfer, C.-M. Lehr und C. Müller geben an, dass kein Interessenkonflikt besteht.
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Vogt, T., Thomas, C., Reichrath, J. et al. Postmenopausaler Lichen planopilaris alias fibrosierende frontotemporale Alopezie Kossard. Hautarzt 69, 134–142 (2018). https://doi.org/10.1007/s00105-017-4118-x
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DOI: https://doi.org/10.1007/s00105-017-4118-x