Clinical and Experimental Dermatology

An unusual case of multiple facial papules? J. B. Powell,1 W. Szczecinska,1 P. Gazzani,1 J. Miller,1 H. Dzwoniakiewicz,1 D. Snead,2 R. Carr3 and A. Ilchyshyn1 Departments of 1Dermatology and 2Histopathology, Worcestershire Royal Hospital, Worcester, UK; and 3Department of Histopathology, Warwick Hospital, Warwick, UK

Clinical findings A 38-year-old woman presented with a 2-year history of multiple asymptomatic facial papules, which had started periorally and had continued to spread over her face. She had also noted progressive loss of her eyebrow hair over 4 years, and loss of the hair on her arms and legs for 1 year. Her scalp was also itchy. She was not postmenopausal and was taking salbutamol inhalers for asthma and valsartan for hypertension. On physical examination, eyebrow loss was noted, along with multiple widespread noninflammatory, skin-coloured facial papules (Fig. 1a). These papules were especially prominent on the nasolabial folds, where a cobblestone and roughened appearance had developed, and on the lateral chin areas (Fig. 1b,c). A localized area of scarring alopecia with perifollicular erythema and scaling was noted at the central frontal hair margin, although the patient had not noticed any hair loss in this area (Fig. 1d). No mucosal involvement or skin lesions were present elsewhere.

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Figure 1 (a) Eyebrow loss with multiple clinically noninflamma-

Histopathological findings Histological examination of a scalp biopsy (Fig. 2a,b) revealed a peri-infundibular lichenoid infiltrate with individual cell necrosis and focal loss of sebaceous units. A biopsy from a representative area on the face (Fig. 2c,d) revealed a focal subtle interface reaction, perifollicular lymphocytic infiltrate and intermittent missing follicles.

tory planar facial papules; (b,c) facial papules prominent on the nasolabial fold and lateral chin areas, where a cobblestone, roughened and lichenified appearance had developed; (d) perifollicular erythema and scaling at the frontal hair margin with scarring alopecia.

What is your diagnosis?

Correspondence: Dr James B. Powell, Department of Dermatology, Worcestershire Royal Hospital, Worcester, WR5 1DD, UK E-mail: [email protected] Conflict of interest: the authors declare that they have no conflicts of interest. Accepted for publication 15 July 2014

ª 2015 British Association of Dermatologists

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Figure 2 Representative biopsies from the scalp (a and b). (a,b) This biopsy was sectioned horizontally at the level of the follicular isth-

mus, and showed striking perifollicular lymphocytic infiltrates and some loss of sebaceous units, with (b) a lichenoid pattern of perifollicular inflammation at the upper stem with individual follicular keratinocyte necrosis and perifollicular concentric fibrosis. (c,d) Representative biopsies from facial papules, showing perifollicular lichenoid lymphocytic infiltrates (arrows) and missing follicles replaced by subtle fibrous scars (arrowheads). Inset in (c) shows lymphocytes in the outer layer of the follicular epithelium with a hint of liquefactive change. Haematoxylin and eosin, original magnification (a,c) 9 50; (b) 9 200; (d) 9 100.

Diagnosis

Frontal fibrosing alopecia (FFA).

Discussion FFA is an idiopathic primary lymphocytic scarring alopecia with distinct clinical features, which is increasingly encountered in dermatological practice. It is still considered a variant of lichen planopilaris, because of their identical histopathological findings. FFA is mostly seen in postmenopausal women, although premenopausal women can be affected. Men make up only a minority of cases. Perfollicular erythema and scaling affecting the frontal hair margin with resultant progressive frontotemporal hairline

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recession are the clinical hallmarks of FFA. The generalized nature of FFA with the involvement of hairbearing sites away from the scalp is now well recognized.1 In a recent multicentre review of 355 patients in Spain with FFA, 80% were found to have eyebrow loss (with hair loss beginning here in 39%) and 14% eyelash loss, while body hair was affected in 24%, axillary and pubic hair in 21% and the beard area in 50% (of affected men).2 The presence and nature of facial papules in FFA has rarely been reported previously. Their noninflammatory nature, prominence in the temporal regions of the face, resultant ‘roughened facial skin’ and their appearance of being ‘keratosis pilaris-like’ have been noted.3,4 Facial papules in FFA show subtle

ª 2015 British Association of Dermatologists

histopathological features of a perifollicular fibrosis with a lymphocytic infiltrate in a lichenoid pattern around the infundibulum and isthmus of the vellus hair follicle, with eventual follicle destruction.4 Similar findings are seen in areas of hair loss from the scalp, eyebrows and body hair in FFA.1,3 Involvement of the facial vellus hairs in FFA, resulting in multiple facial papules, underlines the ability of this disease to affect areas outside of the scalp and the generalized nature of this disease, and is an underrecognized phenomenon. Treatment of FFA is often challenging. As our patient remained symptomatic with clinical features on the scalp of active disease, despite topical and intralesional steroids, we initiated hydroxychloroquine 400 mg daily, which has also been reported to improve the facial skin surface in those with facial papules in FFA.4 It is important to recognize the presence of facial papules in FFA, which may, as in this case, be the presenting feature. Such recognition may help avoid unnecessary investigations, treatments and anxiety regarding their aetiology.

ª 2015 British Association of Dermatologists

Learning points ● FFA is a generalized skin condition in which

multiple facial papules may occur. ● Facial papules may be the presenting feature.

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. -Galv 2 Va~ no an S, Molina-Ruiz AM, Serrano-Falc on C et al. Frontal fibrosing alopecia: a multicenter review of 355 patients. J Am Acad Dermatol 2014; 70: 670–8. 3 Tan KT, Messenger AG. Frontal fibrosing alopecia: clinical presentations and prognosis. Br J Dermatol 2009; 160: 75–9. 4 Donati A, Molina L, Doche I et al. Facial papules in frontal fibrosing alopecia. Arch Dermatol 2011; 147: 1424–7.

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Clinicopathological case

An unusual case of multiple facial papules?

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