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 Table of Contents  
CASE REPORT
Year : 2013  |  Volume : 5  |  Issue : 4  |  Page : 217-219  

Frontal fibrosing alopecia and lupus overlap in a man: Guilt by association?


1 Department of Dermatopathology; St. John's Institute of Dermatology, St. Thomas' Hospital, London, United Kingdom
2 St. John's Institute of Dermatology, St. Thomas' Hospital, London, United Kingdom

Date of Web Publication11-Apr-2014

Correspondence Address:
Catherine M Stefanato
Department of Dermatopathology, St. John's Institute of Dermatology, St. Thomas' Hospital, Westminster Bridge Road, SE1 7EH, London
United Kingdom
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0974-7753.130420

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   Abstract 

Frontal fibrosing alopecia (FFA) is a scarring alopecia, now an accepted subset variant of lichen planopilaris (LPP). Its occurrence in males is rare, with only nine cases reported to date. We describe a case of FFA in a male in association with lupus erythematosus. Multiple biopsies from the scalp, eyebrow and arm showed features consistent with LPP, in keeping with the clinical presentation of FFA. Direct immunofluorescence studies showed a positive lupus band test. Further serological investigation confirmed the presence of antinuclear, anticardiolipin and lupus anticoagulant antibodies. Whilst the findings of lupus erythematosus may be coincidental or a forme fruste of the disease occurring in association with FFA, it is feasible that lupus and LPP may occur as an overlap syndrome. This case underscores the importance of multiple biopsies and the role of direct immunofluorescence in disclosing more than one pathology in the follow-up of patients with scarring alopecia.

Keywords: Frontal fibrosing alopecia, immunofluorescence, lupus erythematosus, males, scarring alopecia


How to cite this article:
Khan S, Fenton DA, Stefanato CM. Frontal fibrosing alopecia and lupus overlap in a man: Guilt by association?. Int J Trichol 2013;5:217-9

How to cite this URL:
Khan S, Fenton DA, Stefanato CM. Frontal fibrosing alopecia and lupus overlap in a man: Guilt by association?. Int J Trichol [serial online] 2013 [cited 2019 Nov 17];5:217-9. Available from: http://www.ijtrichology.com/text.asp?2013/5/4/217/130420


   Introduction Top


Kossard first described frontal fibrosing alopecia (FFA) as a scarring alopecia predominantly affecting postmenopausal women. [1] Subsequently, it was reported to occur in pre-menopausal women. [2]

Clinically, it presents as a symmetrical band of frontotemporal and/or frontoparietal hairline recession, slowly progressive in nature. [1],[2] While scalp alopecia predominates, concomitant loss of hair from the eyebrows and peripheral body sites has been reported. [1],[3] Histopathologically, FFA is an accepted variant of lichen planopilaris (LPP). The occurrence of FFA is rare in men. [4],[5],[6],[7],[8],[9] We describe a male patient with FFA who developed overlap features of lupus.


   Case Report Top


A 46-year-old Caucasian male first presented with a 3 months' history of eyebrow thinning and itching. Past medical history included seborrhoeic dermatitis and rosacea. Examination showed erythematous eyebrows associated with pre-auricular and supra-auricular hair loss, deemed clinically to be non-scarring. One year later, photosensitivity at hair loss sites was noted. A left temple biopsy was consistent with scarring alopecia; direct immunofluorescence study was negative. Two years later, he developed further hair loss of scalp, eyebrows, beard, temples, [Figure 1]a] and forearms [Figure 2]a, and six years later complete loss of sideburns [Figure 1]e.
Figure 1: (a) Fronto-temporal recession and eyebrow loss. (b) Scalp: Perifollicular concentric fi brosis with moderately dense perifollicular lymphoid cell infi ltrate consistent with lichen planopilaris, H and E, ×200. (c) Focal basement membrane zone thickening, periodic acid-Schiff stain, ×400. (d) Direct immunofl uorescence: Bright granular-linear deposition of immunoglobulin IgM along basement membrane zone; also IgG, IgA and fibrin deposition was present consistent with a positive lupus band test ×400. (e) 6 years later: Complete loss of sideburns

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Biopsies from the scalp, eyebrow and arm showed follicular dropout and residual hair follicles with a perifollicular moderately dense lymphoid cell infiltrate with perifollicular fibrosis; consistent with LPP [Figure 1]b, [Figure 2]b and [Figure 2]c] and in keeping with the clinical presentation of FFA. Periodic acid-Schiff stain of the scalp and eyebrow biopsies showed focal areas of basement membrane zone thickening [Figure 1]c; direct immunofluorescence demonstrated a bright, granular-linear deposition of immunoglobulins IgM, IgG, IgA and fibrin along the basement membrane zone, consistent with a positive lupus band test [Figure 1]d. Serological testing confirmed the presence of antinuclear, anticardiolipin, as well as lupus anticoagulant antibodies; however, he was free of systemic symptoms.
Figure 2: (a) Forearms: Loss of body hair. (b) Histopathology from a forearm biopsy shows only one residual intermediate-sized hair follicle, H and E, ×40. (c) Higher magnifi cation showing perifollicular concentric
fi brosis and a perifollicular lymphoid cell infi ltrate consistent with lichen planopilaris, H and E, ×100


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   Discussion Top


[Table 1] lists reports of FFA in men. Ramaswamy et al. [7] described sideburn loss as a feature of FFA, as in our patient. Chen et al. reported a male with associated generalized hair loss, thus reinforcing the concept of expansion of FFA to peripheral body sites. [8] Although our findings of lupus erythematosus may be coincidental or represent a forme fruste of the disease, it is feasible that lupus and LPP may overlap, in a similar fashion to a previously described lupus and lichen planus overlap syndrome. [10]
Table 1: Frontal fi brosing alopecia in men

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Gaffney et al. [11] in their study reported the occurrence of FFA with discoid lupus erythematosus in a 69-year-old Caucasian woman. In contrast to our case, the reported patient was female, had a known background history of lupus, and distribution of the scalp hair loss sites did not overlap. Although LPP and lupus may share similar histopathological findings, direct immunofluorescence was helpful in our case for detecting the presence of lupus overlap.

Whilst this is the ninth case of biopsy-proven FFA in a man, it is unique, as, to our knowledge, it is the first description of FFA with coexisting lupus in the male population.

 
   References Top

1.Kossard S. Postmenopausal frontal fibrosing alopecia. Scarring alopecia in a pattern distribution. Arch Dermatol 1994;130:770-4.  Back to cited text no. 1
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2.Faulkner CF, Wilson NJ, Jones SK. Frontal fibrosing alopecia associated with cutaneous lichen planus in a premenopausal woman. Australas J Dermatol 2002;43:65-7.  Back to cited text no. 2
    
3.Chew AL, Bashir SJ, Wain EM, Fenton DA, Stefanato CM. Expanding the spectrum of frontal fibrosing alopecia: A unifying concept. J Am Acad Dermatol 2010;63:653-60.  Back to cited text no. 3
    
4.Stockmeier M, Kunte C, Sander CA, Wolff H. Kossard frontal fibrosing alopecia in a man [German]. Hautarzt 2002;53:409-11.  Back to cited text no. 4
    
5.Kossard S, Shiell RC. Frontal fibrosing alopecia developing after hair transplantation for androgenetic alopecia. Int J Dermatol 2005;44:321-3.  Back to cited text no. 5
    
6.Nusbaum BP, Nusbaum AG. Frontal fibrosing alopecia in a man: Results of follicular unit test grafting. Dermatol Surg 2010;36:959-62.  Back to cited text no. 6
    
7.Ramaswamy P, Mendese G, Goldberg LJ. Scarring alopecia of the sideburns: A unique presentation of frontal fibrosing alopecia in men. Arch Dermatol 2012;148:1095-6.  Back to cited text no. 7
[PUBMED]    
8.Chen W, Kigitsidou E, Prucha H, Ring J, Andres C. Male frontal fibrosing alopecia with generalised hair loss. Australas J Dermatol Online 29 th Nov 2012.  Back to cited text no. 8
    
9.Dlova NC, Goh CL. Frontal fibrosing alopecia in an African man. Int J Dermatol 2013 [Epub ahead of Print].  Back to cited text no. 9
    
10.Inalöz HS, Chowdhury MM, Motley RJ. Lupus erythematosus/lichen planus overlap syndrome with scarring alopecia. J Eur Acad Dermatol Venereol 2001;15:171-4.  Back to cited text no. 10
    
11.Gaffney DC, Sinclair RD, Yong-Gee S. Discoid lupus alopecia complicated by frontal fibrosing alopecia on a background of androgenetic alopecia. Br J Dermatol 2013;169:217-8.  Back to cited text no. 11
[PUBMED]    


    Figures

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