Frontal fibrosing alopecia

European Journal of Dermatology. Volume 13, Number 2, 203-4, March - April 2003, Cas cliniques

Author(s) : Maria Cristina FIORUCCI, Emmanuel COZZANI, Aurora PARODI, Alfredo REBORA, DiSEM, Section of Dermatology, University of Genoa Viale Benedetto XV, 7, 16132 Genova, Italy.

Summary : 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.

Keywords : alopecia, frontal fibrosing alopecia, postmenopausal alopecia

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ARTICLE

The progressive recession of the frontal hairline is a common event in postmenopausal women and is usually considered a variant of androgenetic alopecia. A rare cicatricial form unrelated to any sign of virilization may be observed, however, and called "postmenopausal frontal fibrosing alopecia" (PFFA). Its nature and pathogenesis are obscure, only a few cases being reported after its original description in 1994 [1-4].

Case report

Case 1

A 65-year-old female had complained of hairline recession for 2 years. She denied hair shedding from the scalp or other hairy areas and taking any medication. She did not show symptoms or cutaneous signs of other diseases, in particular lupus erythematosus. On examination, a symmetrical frontal band of marginal alopecia was observed (Fig. 1). The alopecic skin was uniformly pale contrasting with the rest of the forehead, without erythema or scaling. The follicular orifices were not detectable. The scalp hairs were normal and the pull test was negative. The eyebrows were normal. No cutaneous, mucous membrane and nail lesions were present. All laboratory tests were negative or within normal limits including hormonal and immunologic parameters.

A biopsy from the frontal area was taken and histology showed mild epidermal hyperplasia and hyperkeratosis, reduction in number of hair follicles with dermal fibrosis and a mild perivascular lymphocytic infiltrate (Fig. 2).

Case 2

A 64-year-old woman presented with frontal recession extending to the marginal temporal and parietal areas of the scalp. The manifestation had slowly developed over 3 years, resulting in a symmetrical band of marginal alopecia.

The alopecic skin was frankly cicatricial and both eyebrows were markedly thinned. No signs of lichen planopilaris or androgenetic alopecia were observed. The mucous membranes and the remaining skin were normal.

Routine laboratory tests including full blood cell count, erythrocyte sedimentation rate, liver and thyroid function tests were normal. Biopsy was not granted.

Discussion

PFFA is characterised by a fronto-parietal recession of the hair line resulting in an area of alopecia. The skin is uniformly pale without erythema and scaling and the follicular orifices are not visible. In some cases a thinning of eyebrows is also present. PFFA usually affects post menopausal women, but two cases in pre-menopausal women have also been reported [3, 5].

Only in a few cases does histology show the lymphocytic impingement of the follicle typical of lichen planus pilaris. In most cases, as in our first patient, only a perifollicular lymphocytic infiltrate and a reduced number of hair follicles that are replaced by fibrous tracts can be seen. All laboratory investigations including hormonal profile are negative.

Several forms of alopecia need to be considered in the differential diagnosis, in particular discoid lupus erythematosus, traction alopecia, androgenetic alopecia and lichen planus pilaris. Discoid lupus erythematosus of the scalp shows erythema, hyperkeratosis and atrophy and, in non active forms, peripheral hyperpigmentation. Traction alopecia may produce hair loss localised at the frontal hairline associated with a ragged border and broken hairs of different lengths. Lichen planus pilaris of the scalp presents an asymptomatic patchy alopecia with perifollicular erythema, follicular keratotic plugs and scarring. In addition, classical lichen planus lesions are present on the glabrous skin and mucosae in about 50% of the patients. Androgenetic alopecia usually spares the frontal hairline in women and, when present, produces the typical bitemporal recession as a sign of severe virilization.

No effective treatments for PFFA have been reported, although oral corticosteroids and chloroquine may temporarily curb the course of the disease [2, 3]. Hormone replacement therapy has no effect.

The nature of PFFA remains obscure, but the histopathologic and immunohistochemical features have suggested that it is a variant of lichen planus pilaris with a specific location. On the other hand, its occurrence in the postmenopausal age is no longer distinctive and the term of fibrosing frontal alopecia (FFA) should be preferred.

CONCLUSION

Article accepted on 10/01/2003

REFERENCES

Kossard S. Postmenopausal frontal fibrosing alopecia. Scarring alopecia in a pattern distribution. Arch Dermatol 1994; 130: 770-4.

Camacho Martinez F, Garcia Hernandez MJ, Mazuecos Blanca J. Postmenopausal frontal fibrosing alopecia. Br J Dermatol 1999; 140: 1181-2.

Kossard S, Lee MS, Wilkinson B. Postmenopausal frontal fibrosing alopecia: a frontal variant of lichen planopilaris. J Am Acad Dermatol 1997; 36: 59-66.

Lee WS, Hwang SM, Ahn SK. Frontal fibrosing alopecia in a postmenopausal woman. Cutis 1997; 60: 299-300.

Faulkner C, Wilson N, Jones S. Frontal fibrosing alopecia associated with cutaneous lichen planus in a premenopausal woman. Austral J Dermatol 2002; 45: 65-7.