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Australasian Journal of Dermatology (2014) 55, e37–e39
doi: 10.1111/ajd.12005
BRIEF REPORT
Male frontal fibrosing alopecia with generalised hair loss WenChieh Chen, Evanthia Kigitsidou, Hanna Prucha, Johannes Ring and Christian Andres Department of Dermatology and Allergy, Technische Universität München, Munich, Germany
ABSTRACT Frontal fibrosing alopecia predominantly affects postmenopausal women and is regarded as a variant of lichen planopilaris. Male cases have rarely been reported. Here we describe a 66-year-old man with a typical receding fronto-temporal hair line in a form of scarring alopecia, which shows features of lichen planopilaris in histology. An extensive loss of body hair involving bilateral axillae, limbs and pubic area was also observed. Key words: androgenetic alopecia, frontal fibrosing alopecia, lichen planopilaris.
INTRODUCTION Frontal fibrosing alopecia (FFA), first described by Kossard and colleagues in 1994, is a form of scarring alopecia involving predominantly white postmenopausal women.1–3 In addition to the typical frontal hair recession, eyebrow loss has been found in 75 per cent of these patients.2 A clinically non-scarring hair loss involving the eyelashes, axillae and extremities has also been observed with different prevalence rates.2–4 A fibrosing process in histopathology has been documented in later reports.1,4 FFA is considered by many authors to be a variant of lichen planopilaris (LPP).1–3 So far very few male patients with FFA have been reported.2,5–8 Here we describe another male patient with FFA and generalised hair loss.
CASE REPORT A 66-year-old, otherwise normal healthy man came to us in February 2011 complaining of noticeable hair loss of the scalp in the last 6 months, with an accelerating rapid regression of the fronto-temporal hair line upon pre-
Correspondence: Dr WenChieh Chen, Department of Dermatology and Allergy, Technische Universität München, Munich, Germany. Email: [email protected] WenChieh Chen, MD. Evanthia Kigitsidou, MD. Hanna Prucha, MD Johannes Ring, MD, PhD.. Christian Andres, MD. Conflict of interest: none Submitted 23 August 2012; accepted 11 October 2012.
existing long-standing androgenetic alopecia (Fig. 1a). His personal and familial medical histories were unremarkable and laboratory examinations, including thyroid function, showed normal results. Skin examinations revealed the effacement and disappearance of most hair orifices in the affected fronto-temporal scalp as well as perifollicular erythema and scaling in several hair follicles. Hair loss was also observed in the eyebrows, pubis, bilateral axillae and four extremities (Fig. 2). Changes in the skin elsewhere, oral mucosa or nails were inconspicuous. A histopathological examination on an erythematous inflammatory papule from the scalp showed a dense, lichenoid lymphocytic infiltrate attacking the follicular epithelium (interface dermatitis) with apoptotic basal cells accompanied by lymphocytes, predominantly seen in the isthmus and bulge region, which conforms to the histological diagnosis of LPP (Fig. 1b). The patient was initially treated with oral prednisolone beginning at 1 mg/kg and tapering out in 2 weeks, in combination with a topical application of 0.05% clobetasol propionate foam for 3 months. The inflammation was no longer apparent after treatment and further hair loss was stopped. Subsequently a topical treatment with 5% minoxidil was tried on the scalp for 6 months to stimulate the hair regrowth but without significant effect. The follow up in December 2011 showed a stable condition without further regression of the fronto-temporal hair line. In a recent telephonic follow up in October 2012 the patient reported a slow relapse of the disease since middle 2012. No regrowth of the lost body hair has so far been observed.
DISCUSSION In our patient, the fronto-temporal band-like distribution of scarring alopecia and the lichenoid pattern of lymphocytic infiltration in histology make the diagnosis of FFA most likely. Our case is conspicuous for an extensive body hair loss involving axillae, limbs and pubic area, which has so far been described only in women.4 FFA is rarely observed in men. The diagnosis may be overlooked, especially when FFA is superimposed on pre-existing androgenetic alopecia, which is very common in elderly men. Among the six
Abbreviations: FFA LPP
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
frontal fibrosing alopecia lichen planopilaris
e38
W Chen et al.
Figure 1 A 66-year-old man with frontal fibrosing alopecia superimposed on pre-existing androgenetic alopecia. (a) A band-like, glossy sclerosing alopecia can be seen in the vertex area. (b) Histology from an inflamed hair follicle showed typical features of lichen planopilaris with an interface dermatitis of the follicular epithelium with a dense lichenoid lymphocytic infiltrate predominantly in the isthmus and bulge region.
Figure 2
A clinical non-cicatricial hair loss is also observed in (a) the eyebrows, (b) pubic areas, (c) axillae and (d) lower extremities.
patients reported so far, including ours, the diagnosis was confirmed in five patients with typical histological changes of LPP.2,5–8 Coexistence of FFA with androgenetic alopecia was found in five patients, while mucocutaneous lichen planus was absent in three patients under examination. The age range of the patients was between 44 and 75 years. The time duration between disease onset and diagnosis was between 6 months and 5 years. Clinically the peripheral body hair loss looks non-scarring, probably because of the
low density of hair follicles in these body areas.4 However, the histology of a few skin biopsies taken from the affected upper extremities showed perifollicular lymphocytic infiltrates, thickening of the granular cell layers as well as basal vacuolar degeneration, indicating the occurrence of LPP in the peripheral hair follicles.1,4 The histological findings of dermal fibrous tract associated with residual portion of arrector pili muscle may suggest a scarring process in some of the involved areas.1
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
Male frontal fibrosing alopecia Not much is known about the pathogenesis and course of FFA in male patients. LPP or FFA following hair transplantation or face-lift surgery has been described both in men and women.6,9 The treatment of FFA is challenging and the various treatment modalities described to date have produced ambiguous effects, especially in promoting hair regrowth.1–4 The most commonly recommended treatment is with hydroxychloroquine 400 mg daily for 6 months. In our patient, the combination use of a short-term systemic steroid treatment with a subsequent 3-month topical potent steroid treatment seemed to stop disease progression effectively for more than 6 months. A fluctuation of the disease course cannot be ruled out. More evidence is needed to confirm whether an early aggressive intervention can achieve an optimal prognosis.
2.
3.
4.
5.
6.
7.
8.
REFERENCES 1.
Chew AL, Bashir SJ, Wain EM et al. Expanding the spectrum of frontal fibrosing alopecia: a unifying concept. J. Am. Acad. Dermatol. 2010; 63: 653–60.
9.
e39
Samrao A, Chew AL, Price V. Frontal fibrosing alopecia: a clinical review of 36 patients. Br. J. Dermatol. 2010; 163: 1296– 300. Conde Fernandes I, Selores M, Machado S. Frontal fibrosing alopecia: a review of eleven patients. Eur. J. Dermatol. 2011; 21: 750–2. Armenores P, Shirato K, Reid C et al. Frontal fibrosing alopecia associated with generalized hair loss. Australas. J. Dermatol. 2010; 51: 183–5. Nusbaum BP, Nusbaum AG. Frontal fibrosing alopecia in a man: results of follicular unit test grafting. Dermatol. Surg. 2010; 36: 959–62. Kossard S, Shiell RC. Frontal fibrosing alopecia developing after hair transplantation for androgenetic alopecia. Int. J. Dermatol. 2005; 44: 321–3. Stockmeier M, Kunte C, Sander CA et al. [Kossard frontal fibrosing alopecia in a man]. Hautarzt 2002; 53: 409–11. (In German.) Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch. Dermatol. 2000; 136: 205–11. Chiang YZ, Tosti A, Chaudhry IH et al. Lichen planopilaris following hair transplantation and face-lift surgery. Br. J. Dermatol. 2012; 166: 666–70.
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
Australasian Journal of Dermatology (2014) 55, e37–e39
doi: 10.1111/ajd.12005
BRIEF REPORT
Male frontal fibrosing alopecia with generalised hair loss WenChieh Chen, Evanthia Kigitsidou, Hanna Prucha, Johannes Ring and Christian Andres Department of Dermatology and Allergy, Technische Universität München, Munich, Germany
ABSTRACT Frontal fibrosing alopecia predominantly affects postmenopausal women and is regarded as a variant of lichen planopilaris. Male cases have rarely been reported. Here we describe a 66-year-old man with a typical receding fronto-temporal hair line in a form of scarring alopecia, which shows features of lichen planopilaris in histology. An extensive loss of body hair involving bilateral axillae, limbs and pubic area was also observed. Key words: androgenetic alopecia, frontal fibrosing alopecia, lichen planopilaris.
INTRODUCTION Frontal fibrosing alopecia (FFA), first described by Kossard and colleagues in 1994, is a form of scarring alopecia involving predominantly white postmenopausal women.1–3 In addition to the typical frontal hair recession, eyebrow loss has been found in 75 per cent of these patients.2 A clinically non-scarring hair loss involving the eyelashes, axillae and extremities has also been observed with different prevalence rates.2–4 A fibrosing process in histopathology has been documented in later reports.1,4 FFA is considered by many authors to be a variant of lichen planopilaris (LPP).1–3 So far very few male patients with FFA have been reported.2,5–8 Here we describe another male patient with FFA and generalised hair loss.
CASE REPORT A 66-year-old, otherwise normal healthy man came to us in February 2011 complaining of noticeable hair loss of the scalp in the last 6 months, with an accelerating rapid regression of the fronto-temporal hair line upon pre-
Correspondence: Dr WenChieh Chen, Department of Dermatology and Allergy, Technische Universität München, Munich, Germany. Email: [email protected] WenChieh Chen, MD. Evanthia Kigitsidou, MD. Hanna Prucha, MD Johannes Ring, MD, PhD.. Christian Andres, MD. Conflict of interest: none Submitted 23 August 2012; accepted 11 October 2012.
existing long-standing androgenetic alopecia (Fig. 1a). His personal and familial medical histories were unremarkable and laboratory examinations, including thyroid function, showed normal results. Skin examinations revealed the effacement and disappearance of most hair orifices in the affected fronto-temporal scalp as well as perifollicular erythema and scaling in several hair follicles. Hair loss was also observed in the eyebrows, pubis, bilateral axillae and four extremities (Fig. 2). Changes in the skin elsewhere, oral mucosa or nails were inconspicuous. A histopathological examination on an erythematous inflammatory papule from the scalp showed a dense, lichenoid lymphocytic infiltrate attacking the follicular epithelium (interface dermatitis) with apoptotic basal cells accompanied by lymphocytes, predominantly seen in the isthmus and bulge region, which conforms to the histological diagnosis of LPP (Fig. 1b). The patient was initially treated with oral prednisolone beginning at 1 mg/kg and tapering out in 2 weeks, in combination with a topical application of 0.05% clobetasol propionate foam for 3 months. The inflammation was no longer apparent after treatment and further hair loss was stopped. Subsequently a topical treatment with 5% minoxidil was tried on the scalp for 6 months to stimulate the hair regrowth but without significant effect. The follow up in December 2011 showed a stable condition without further regression of the fronto-temporal hair line. In a recent telephonic follow up in October 2012 the patient reported a slow relapse of the disease since middle 2012. No regrowth of the lost body hair has so far been observed.
DISCUSSION In our patient, the fronto-temporal band-like distribution of scarring alopecia and the lichenoid pattern of lymphocytic infiltration in histology make the diagnosis of FFA most likely. Our case is conspicuous for an extensive body hair loss involving axillae, limbs and pubic area, which has so far been described only in women.4 FFA is rarely observed in men. The diagnosis may be overlooked, especially when FFA is superimposed on pre-existing androgenetic alopecia, which is very common in elderly men. Among the six
Abbreviations: FFA LPP
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
frontal fibrosing alopecia lichen planopilaris
e38
W Chen et al.
Figure 1 A 66-year-old man with frontal fibrosing alopecia superimposed on pre-existing androgenetic alopecia. (a) A band-like, glossy sclerosing alopecia can be seen in the vertex area. (b) Histology from an inflamed hair follicle showed typical features of lichen planopilaris with an interface dermatitis of the follicular epithelium with a dense lichenoid lymphocytic infiltrate predominantly in the isthmus and bulge region.
Figure 2
A clinical non-cicatricial hair loss is also observed in (a) the eyebrows, (b) pubic areas, (c) axillae and (d) lower extremities.
patients reported so far, including ours, the diagnosis was confirmed in five patients with typical histological changes of LPP.2,5–8 Coexistence of FFA with androgenetic alopecia was found in five patients, while mucocutaneous lichen planus was absent in three patients under examination. The age range of the patients was between 44 and 75 years. The time duration between disease onset and diagnosis was between 6 months and 5 years. Clinically the peripheral body hair loss looks non-scarring, probably because of the
low density of hair follicles in these body areas.4 However, the histology of a few skin biopsies taken from the affected upper extremities showed perifollicular lymphocytic infiltrates, thickening of the granular cell layers as well as basal vacuolar degeneration, indicating the occurrence of LPP in the peripheral hair follicles.1,4 The histological findings of dermal fibrous tract associated with residual portion of arrector pili muscle may suggest a scarring process in some of the involved areas.1
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
Male frontal fibrosing alopecia Not much is known about the pathogenesis and course of FFA in male patients. LPP or FFA following hair transplantation or face-lift surgery has been described both in men and women.6,9 The treatment of FFA is challenging and the various treatment modalities described to date have produced ambiguous effects, especially in promoting hair regrowth.1–4 The most commonly recommended treatment is with hydroxychloroquine 400 mg daily for 6 months. In our patient, the combination use of a short-term systemic steroid treatment with a subsequent 3-month topical potent steroid treatment seemed to stop disease progression effectively for more than 6 months. A fluctuation of the disease course cannot be ruled out. More evidence is needed to confirm whether an early aggressive intervention can achieve an optimal prognosis.
2.
3.
4.
5.
6.
7.
8.
REFERENCES 1.
Chew AL, Bashir SJ, Wain EM et al. Expanding the spectrum of frontal fibrosing alopecia: a unifying concept. J. Am. Acad. Dermatol. 2010; 63: 653–60.
9.
e39
Samrao A, Chew AL, Price V. Frontal fibrosing alopecia: a clinical review of 36 patients. Br. J. Dermatol. 2010; 163: 1296– 300. Conde Fernandes I, Selores M, Machado S. Frontal fibrosing alopecia: a review of eleven patients. Eur. J. Dermatol. 2011; 21: 750–2. Armenores P, Shirato K, Reid C et al. Frontal fibrosing alopecia associated with generalized hair loss. Australas. J. Dermatol. 2010; 51: 183–5. Nusbaum BP, Nusbaum AG. Frontal fibrosing alopecia in a man: results of follicular unit test grafting. Dermatol. Surg. 2010; 36: 959–62. Kossard S, Shiell RC. Frontal fibrosing alopecia developing after hair transplantation for androgenetic alopecia. Int. J. Dermatol. 2005; 44: 321–3. Stockmeier M, Kunte C, Sander CA et al. [Kossard frontal fibrosing alopecia in a man]. Hautarzt 2002; 53: 409–11. (In German.) Zinkernagel MS, Trüeb RM. Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch. Dermatol. 2000; 136: 205–11. Chiang YZ, Tosti A, Chaudhry IH et al. Lichen planopilaris following hair transplantation and face-lift surgery. Br. J. Dermatol. 2012; 166: 666–70.
© 2012 The Authors Australasian Journal of Dermatology © 2012 The Australasian College of Dermatologists
