Frontal Fibrosing Alopecia and Lupus Overlap in a Man: Guilt by Association?

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.

INTRODUCTION

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

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 1a] and forearms [Figure 2a], and six years later complete loss of sideburns [Figure 1e].

Figure 1.

(a) Fronto-temporal recession and eyebrow loss. (b) Scalp: Perifollicular concentric fibrosis with moderately dense perifollicular lymphoid cell infiltrate consistent with lichen planopilaris, H and E, ×200. (c) Focal basement membrane zone thickening, periodic acid-Schiff stain, ×400. (d) Direct immunofluorescence: 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

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 magnification showing perifollicular concentric fibrosis and a perifollicular lymphoid cell infiltrate consistent with lichen planopilaris, H and E, ×100

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 [Figures 1b, 2b and 2c] 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 1c]; 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 1d]. Serological testing confirmed the presence of antinuclear, anticardiolipin, as well as lupus anticoagulant antibodies; however, he was free of systemic symptoms.

DISCUSSION

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 fibrosing alopecia in men

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.