Frontal fibrosing alopecia L66.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 09.08.2022

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Synonym(s)

Alopecia frontal fibrosis; FAPD; frontal fibrosing alopecia (e); Kossard's disease; Kossard syndrome; Postmenopausal frontal fibrosing alopecia; PPFA

History
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Kossard, 1994

Definition
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Postmenopausal-onset, circumscribed, band-like, symmetric, fibrosing form of scarring alopecia (irreversible) in the frontotemporal hairline in women with lymphoid-histiocytic infiltrates around the hair follicles. Often associated with rarefication of the eyebrows. Less commonly, men suffer from this clinical picture. Frequently, this form of chronic insidious alopecia is associated with extremity-emergent keratosis pilaris (friction skin), which is clinically prominent in adolescence and early adulthood.

The Frontal Fibrosing Alopecia - FFA- Severity Index is used for standardized assessment of the severity of the clinical picture. The score includes spread, involvement of body and facial hair, skin involvement, mucosa and nail involvement.

If >/= 4 points with typical criteria for frontal fibrosing alopecia, the diagnosis should be made.

The frontal recession of the forehead-hairline with loss of hair follicle ostia counts with 2 points, the positive biopsy in one of the affected areas: frontal or temproal scalp area resp. eyebrows also counts with 2 points, 1 point each for the loss of at least 50% of the eyebrows and 1 point for follicular erythema on the frontal scalp, also 1 point for perifollicular scalp hyperkeratosis or dandruff on the frontal scalp.

Occurrence/Epidemiology
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Almost exclusively women. In a larger collective about 3% men were described.

Etiopathogenesis
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Unsolved. A variant of lichen planus follicularis or a partial manifestation of a so-called keratosis- rubra-pilaris syndrome is being discussed.

Manifestation
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Beginning of clinically noticeable symptoms between 56 and 60 years of age (in a larger collective, the mean age at the time of diagnosis was 61 years, ranging from 23 - 86 years).

Clinical features
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Mostly imperceptible onset without subjective symptoms: receding frontal hairline, spreading to parietal and occipital regions.

Effluvium is usually not noticed.

The disease only becomes evident when the chronic creeping receding of the frontal or temporal hairline becomes conspicuous (image comparison with earlier).

The clinical picture of the band-shaped fronto-temporal alopecia can be superimposed by an androgenetic effluvium (now the hair problem is noticed!).

Band-shaped, frontal or frontotemporal also exclusively temporal hairlessness (alopecia);

The clinical picture ranges from mild, grade I (frontal hairline recession of <1.0cm) to grade V (frontal hairline recession of >7 cm). The grade V condition is also referred to as "clown alopecia".

No hair follicles detectable in the affected aral;

Skin in affected areas markedly lighter, less tanned.

Discrete perifollicular redness or follicular keratotic papules in adjacent hair area.

Associated disorders: In most cases, alopecia is associated with ulerythema ophryogenes as well as keratosis follicularis.

Remark: Often the keratosis follicularis is not noticed anymore, because in the advanced age of the patient it does not show its typical expression (rubbing iron skin) but only "as not disturbing but rather welcome, hairless, smooth areas" of the extensor extremities (indications: no hair at all on the extensor sides of the forearms).

Clinically, 3 different patterns of regression of the forehead-hair boundary are distinguished:

  • Type I: Band-like
  • Type II: further foci of alopecia behind the forehead-hair line
  • Type III: preserved frontal hairline, band-like pattern behind it (pseudo-fringe sign).

Histology
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Perifollicular lymphocytic infiltrate and perifollicular fibrosis at the level of the isthmus and the infundibulum Vacuole degeneration of basal follicular keratinocytes and single cell necrosis in the follicular epithelium.

Differential diagnosis
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Therapy
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Most unsatisfactory! No causal therapy known, insofar symptomatic therapy approaches.

General therapy
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Trial with minoxidil solution. Antiandrogens such as finasteride were successful in half of the patients treated in this way. The most successful therapy with > 62 % proved to be Dutasteride®.

Radiation therapy
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In individual cases, little convincing success with UVB irradiation was described.

Internal therapy
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Trial with oral retinoids .

Remember! Search for Lichen planus stigmata! Finasteride

2.0-5.0 mg/day p.o. In individual cases success with glucocorticosteroids, Ciclosporin A, Azathioprine, Thalidomide, Mycophenolatmofetil has been described.

Progression/forecast
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Chronic course for years or even decades.

Note(s)
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The entity of the clinical picture remains controversial.

Probably partial manifestation of the keratosis pilaris syndrome(ulerythema ophryogenes, keratosis pilaris, alopecia).

It can be assumed that the clinical picture also occurs in men, but is not diagnosed due to the superimposed androgenetic alopecia.

Literature
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  1. Banka N et al (2014) Frontal fibrosing alopecia: a retrospective clinical review of 62 patients with treatment outcome and long-term follow-up. Int J Dermatol 53:1324-1330

  2. Boms S, Gambichler T (2005) Postmenopausal frontal fibrosing alopecia (Kossard) Akt Dermatol 31: 30-32.
  3. Kossard S (1994) Postmenopausal frontal fibrosing alopecia. Arch Dermatol 130: 770-774
  4. Kossard S, Lee MS, Wilkinson B (1997) Postmenopausal frontal fibrosing alopecia: a frontal variant of lichen planopilaris. J Am Acad Dermatol 36: 59-66.
  5. MacDonald A et al (2012) Frontal fibrosing alopecia: a review of 60 cases. J Am Acad Dermatol 67:955-961

  6. Pérez-Rodríguez IM et al (2013) Hyperpigmentation following Treatment of Frontal Fibrosing Alopecia. Case Rep Dermatol 23:357-362
  7. Samrao A et al (2010) Frontal fibrosing alopecia: a clinical review of 36 patients. Br J Dermatol 163:1296-1300
  8. Tosti A et al (2004) Frontal fibrosing alopecia in postmenopausal women. J Am Acad Dermatol 52: 55-60.
  9. Tremezaygues L et al (2012) Frontal fibrosing alopecia with androgenetic pattern. Dermatologist 63: 411-414
  10. Vañó-Galván S et al.(2014)Frontal fibrosing alopecia: a multicenter review of 355 patients. J Am Acad Dermatol 70:670-678.
  11. Wagner G et al.(2016) Frontal fibrosing alopecia Kossard. Dermatologist 67:891-896.
  12. Blume-Peytavi U et al (2022) Frontal fibrosing alopecia - current knowledge. Dermatologist 73: 344-352
  13. Pindado-Ortega C et al (2021) Effectiveness of dutasteride in a large series of patients with frontal fibrosing alopecia in real clinical practice. J Am Acad Dermatol. 84: 1285-1294

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 09.08.2022