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Frontal fibrosing alopecia.

Fiorucci MC, Cozzani E, Parodi A, Rebora A

Eur J Dermatol 2003 Mar-Apr;13(2):203-4.
DiSEM, Section of Dermatology, University of Genoa Viale Benedetto XV, 7, Italy. FAU - Fiorucci, Maria Cristina
PMID: 12695141 [PubMed - indexed for MEDLINE]

Two cases of frontal fibrosing alopecia in post menopausal women, one of them biopsy-proven, are reported. Both women showed a progressive marginal alopecia and none had multifocal areas of involvement typical of lichen planopilaris nor the mucosal or cutaneous lesions of lichen planus. However, scalp biopsy specimens from the frontal hair margin showed perifollicular fibrosis and lymphocytic inflammation. Because the histologic findings are indistinguishable from those seen in lichen planopilaris, frontal fibrosing alopecia is considered a variant of lichen planus pilaris with a particular specific localization and usually onset in the postmenopausal age.

Lichen planopilaris simulating postmenopausal frontal fibrosing alopecia (Kossard)]

Trueb RM, Torricelli R

Hautarzt 1998 May;49(5):388-91.
Dermatologische Klinik, Universitatsspital Zurich. FAU - Trueb, R M
PMID: 9642560 [PubMed - indexed for MEDLINE]

A 68-year old woman presented with a frontal fibrosing alopecia and lesions of the buccal mucous membranes typical for lichen planus. Postmenopausal frontal fibrosing alopecia (PFFA) has recently been described by Kossard as a distinct entity characterized by progressive recession of the frontotemporal and parietal hair margins leading to permanent alopecia in the form of a symmetrical band-like area of scanning in postmenopausal women. The histology (perifollicular lymphocytic infiltration and fibrosis, increase of apoptosis of hair follicle keratinocytes) is indistinguishable from that of lichen planopilaris. The localization and age- and sex-related characteristics of PFFA are not sufficient to delineate it as a discrete entity from lichen planopilaris. Our observation of oral lichen planus in a postmenopausal woman with frontal fibrosing alopecia points to the possibility that PFFA actually may represent a variant of lichen planopilaris with a predilection for the frontotemporal hairline. Other variants of lichen planopilaris include multifocal lichen planopilaris, disseminated lichen planopilaris (Lassueur-Graham-Little syndrome), lichenoid pseudopelade, and any combination of these ("mixed type"). An effective therapy of PFFA is not known. Also, treatment of lichen planopilaris forms in which fibrosis predominates over inflammation is similarly problematic, but the natural course of these diseases seems to be self-limited.

Postmenopausal frontal fibrosing alopecia: a frontal variant of lichen planopilaris.

Kossard S, Lee MS, Wilkinson B

J Am Acad Dermatol 1997 Jan;36(1):59-66.
Skin and Cancer Foundation Australia, NSW, Australia. FAU - Kossard, S
PMID: 8996262 [PubMed - indexed for MEDLINE]

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.

Postmenopausal frontal fibrosing alopecia. Scarring alopecia in a pattern distribution.

Kossard S

Arch Dermatol 1994 Jun;130(6):770-4.
Skin and Cancer Foundation, Sydney, Australia. FAU - Kossard, S
PMID: 8002649 [PubMed - indexed for MEDLINE]

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.

Postmenopausal frontal fibrosing alopecia: 20 cases]

Vaisse V, Matard B, Assouly P, Jouannique C, Reygagne P

Ann Dermatol Venereol 2003 Jun-Jul;130(6-7):607-10.
Unite de Dermatologie, Hopital Tenon, 4, rue de la Chine, 75020 Paris. FAU - Vaisse, V
PMID: 13679696 [PubMed - indexed for MEDLINE]

INTRODUCTION: Postmenopausal frontal fibrosing alopecia is a scarring alopecia that has been recently described by Kossard in 1994 and rarely reported in the literature. OBSERVATIONS: We have conducted a retrospective clinical and histological study of a series of 20 cases of postmenopausal frontal fibrosing alopecia. The results, in agreement with those in the literature, revealed the stereotyped nature of this entity. DISCUSSION: All the clinical and histological data suggest that postmenopausal frontal fibrosing alopecia is a clinical form of lichen planopilaris occurring in elderly women. As with lichen planopilaris its physiopathology is unknown. However, in analogy with other lichen-like dermatoses, a lymphocyte T-mediated autoimmune reaction appears to play a predominant part in its genesis.

Der Hautarzt
Verlag: Springer-Verlag HeiArtikel
delberg
ISSN: 0017-8470 (Paper) 1432-1173 (Online)
DOI: 10.1007/s001050050771
Heft: Band 49, Nummer 6

Datum: Juni 1998
Seiten: 462 - 472
„Vernarbende” Alopezien
Anmerkungen zur Klassifikation, Differentialdiagnose und Pathobiologie

Barbara Hermes A1 and Ralf Paus A1

A1 Dermatologische Universitätsklinik und Poliklinik der Charité (Direktor: Prof. Dr. W. Sterry), Humboldt-Universität zu Berlin

Auszug:

Zusammenfassung

Vernarbende Alopezien mit irreversiblem Follikeluntergang stellen den Endzustand zahlreicher unterschiedlicher Krankheiten dar. Gebräuchliche Klassifikationen können pathogenetische Kriterien nicht zufriedenstellend berücksichtigen, da bisher unklar ist, durch welche Mechanismen im einzelnen der bleibende Follikelverlust bedingt ist. Am Beispiel von Sonderformen (Lichen planopilaris, Pseudopelade) werden bisherige Bemühungen um nosologische Zuordnung, aber auch die Grenzen geltender Klassifikationen und diagnostischer Möglichkeiten kritisch gewürdigt. Eine neue Einteilung der “vernarbenden” Alopezien, bei denen jedoch Vernarbungszeichen keineswegs zwingend sind, wird gefordert, die auf jüngeren Erkenntnissen zur Biologie und Pathologie von Haarfollikel, Haarzyklus und Bindegewebe aufbaut. Wesentlich für das Überleben des Follikels nach Traumatisierung erscheinen Regenerations- und Funktionsfähigkeit sowohl der epithelialen Follikelstammzellen in Höhe des Haarwulstes als auch der hochspezialisierten Fibroblasten der dermalen Papille sowie die molekulare Kommunikation zwischen diesen beiden Zellpopulationen. Die Erforschung vernarbender Alopezien sollte eine Definition pathologischer Prozesse anstreben, die auf den genannten Ebenen zu einer irreversiblen Follikelschädigung führen, um Voraussetzungen für neue therapeutische Optionen (z.B. Stammzellprotektiva, -stimulanzien) zu schaffen.