Flushing episodes in the context of frontal fibrosing alopecia with facial papules

Abstract

Frontal fibrosing alopecia (FFA) is a progressive frontotemporal hairline recession with eyebrow loss. Facial papules are present in up to 14% of FFA cases and can start with facial flushes. Nevertheless, these flushes are commonly associated with rosacea, a much more prevalent disease. In this case, a woman with FFA had, at first, clinical and histopathological findings of rosacea and was treated with ivermectin 1% cream with no improvement. She returned reporting new papules in the frontal region, reaching the frontotemporal hairline. Trichoscopy of the scalp showed mild perifollicular erythema and follicular scale. The new skin biopsy was compatible with FFA, and oral finasteride (5 mg/day) and hydroxychloroquine were introduced to stabilise the disease.

Background

This case describes an uncommon presentation of a dermatological disease, sometimes initially misdiagnosed as rosacea. It reinforces the necessity of a thorough physical examination, including trichoscopy, preceding the skin biopsy. It also outlines the importance of reuniting all clinical signs and symptoms into one diagnosis that better encompasses most of them, instead of misdiagnosing with a more prevalent disease that does not embrace every condition.

Case presentation

A 38-year-old woman reported eyebrow shedding, persistent facial erythema and small papules that had started 6 months before the appointment and had progressed since then. She also described previous frequent episodes of facial flushing that worsened with alcohol intake, stress situations and spicy food ingestion. She was being treated for rosacea by another dermatologist with moisturising lotions, facial soap and sunscreen. She denied cosmetic treatments and concomitant hair shedding. Her family history was negative for any immunological or autoimmune disease. Her physical examination revealed persistent facial erythema, mild eyebrow thinning and facial micropapules (figure 1). No relevant hair recession was noticeable at that time. The trichoscopy of her eyebrow showed no signs of inflammation and no other abnormality besides hair miniaturisation. Clinically, the facial erythema and papules, without relevant hair commitment, endorsed the diagnosis of rosacea. Still, the disease’s rapid progression motivated us to order blood tests and perform a skin biopsy of one facial papule. Six weeks later, she returned with worsening of the eyebrow shedding, more evident facial papules and beginning of the frontotemporal hairline recession. Trichoscopy of the scalp showed mild perifollicular erythema and follicular scale (figure 2). Trichoscopy of the eyebrow was, once more, inconclusive, with no signs of evident follicular commitment. Therefore, we decided to perform another biopsy, taking facial papules and scalp tissue.

Figure 1.

Frontal fibrosing alopecia patient presenting facial papules and persistent erythema. Also note the thinning of the eyebrows and the frontotemporal hairline recess.

Figure 2.

Frontal fibrosing alopecia: dermoscopy of the frontotemporal hairline showing conoid scales around hair follicles and diffuse erythema, 20×.

Investigations

Blood tests came negative for antinuclear antibodies and other rheumatological markers. Blood cell count and thyroid function were normal. Skin biopsy was first performed over a papular area. It showed lymphocytic infiltrate around blood vessels, invading skin appendages, dilated follicle with parakeratotic keratin and presence of Demodex, reinforcing the diagnosis of rosacea. At her second visit, a histopathologcal study of the facial papule (figure 3) revealed a commitment of vellus follicles, with a patchy lichenoid lymphocytic infiltrate blurring the superficial portion of the follicle and also the transition with the inferior portion. Some necrotic keratinocytes were present along with the basal layer. There was a marked decrease of follicular density with numerous fibrotic tracts (figure 4). At the level of the transition between the inferior portion and the isthmus of several follicles, the outer layer was permeated by lymphocytes, presented some necrotic keratinocytes in individual units and was surrounded by perifollicular concentric myxoid fibroplasia and lymphocytic inflammatory infiltrate, corroborating the diagnosis of frontal fibrosing alopecia (FFA).

Figure 3.

Frontal fibrosing alopecia, scalp. Marked decrease in the density of hair follicles with many fibrotic tracts. The remaining hair follicles present permeation of the outer root by lymphocytes with keratinocyte necrosis and are surrounded by concentric fibroplasia and lymphocytes. H&E (horizontal sections), 40×.

Figure 4.

Frontal fibrosing alopecia, facial papule. Lichenoid lymphocytic inflammatory infiltrate around a vellus hair. H&E (vertical sections), 200×.

Differential diagnosis

Initially, the clinical history of facial flushes and persistent erythema led us to confirm the previous diagnosis of rosacea. Nevertheless, eyebrow shedding and rapidly progressing facial papules were not common in this diagnosis, making us think of lichen planopilaris or granulomatous rosacea, motivating skin biopsy and blood work. At her second visit, with such hair loss and typical trichoscopy, FFA with facial papules/lichen planopilaris seemed the most reasonable diagnosis, reuniting all signs and symptoms, and confirmed afterwards with new biopsy specimens from the hairline and facial papules.

Treatment

At first, the patient was treated with ivermectin 1% cream, sunscreen and moisturising facial lotions. After the appropriate diagnosis, 5-alpha-reductase inhibitor (finasteride 5 mg/day) and oral hydroxychloroquine (400 mg/day) were prescribed.¹

Outcome and follow-up

Up to now, 1 year later, the disease is neither progressing nor regressing, with persistent facial papules, facial erythema, mild eyebrow loss and frontotemporal hairline recession of 1 cm. The patient has been submitted to two sessions of erbium laser and two retinoic acid peels, achieving a slight improvement of the rough aspect of the skin.

Discussion

FFA is a kind of scarring alopecia believed to be a spectrum of lichen planopilaris due to their similar histopathological findings in scalp biopsies,² which are: markedly reduced hair density, absence of vellus hair and reduction or complete absence of both arrector pili muscle and sebaceous glands of involved follicles, lichenoid lymphoid involvement of the superior portion of the strand and myxoid concentric fibroplasia.³

Facial papules were first described as ‘keratosis-pilaris-like papules’ over the forehead and cheeks,^{4 5} similar to the lesions described in the trunk and extremities of patients with Graham-Little-Piccardi-Lasseur^{1 6} and then elucidated by Donati et al in 2011 as being facial vellus hair involvement of the disease, with inflamed vellus follicles presenting lichenoid reaction around the follicular infundibulum and isthmus, and perifollicular fibrosis, similar to the findings of terminal hairs in scalp biopsies.⁷ Recently, Pirmez et al reviewed 13 biopsy specimens collected from seven patients with FFA and facial papules and found prominent sebaceous glands, dilated sebaceous ducts, reduction and fragmentation of the elastic fibres (particularly around sebaceous lobules). Vellus hair involvement was seen in only two samples.⁸ Nevertheless, the pathologists know that it can be sometimes tricky to localise inflammatory infiltrate around hair follicles, especially around vellus hair, being necessary to have both horizontally and vertically oriented samples. There was a lichenoid infiltrate around isthmus of vellus hair in our patient, with no significant alteration in sebaceous gland, probably due to an early-stage diagnosis.

In our case, the first skin biopsy revealed perivascular and periadnexal inflammatory infiltrate, which is found both in rosacea and in lichen planopilaris. However, the dilated follicle with parakeratotic keratin and Demodex presence corroborated the diagnosis of rosacea at that moment. Despite the low-grade frontotemporal hairline recession and mild eyebrow thinning, she also presented facial papules, which are related to severe FFA forms.

Based on the history of flushing and histological findings, we believed, at first, that this was a case of rosacea associated with FFA. Still, we cannot rule out the possibility of all the signs and symptoms being part of FFA since the beginning. Similar to our case, Lopez-Pestaña et al described, in 2015, patients who were previously diagnosed with rosacea, progressing with facial papules of FFA afterwards.⁹ Although different diagnoses might be present in one patient, we must always make an effort to reunite symptoms into one single syndrome whenever possible.

This case highlights the importance of a thorough physical examination and dermoscopy of the hair follicles as a valuable tool for early diagnosis of FFA before biopsy and calls attention to the fact that facial papules can represent the association of rosacea and FFA or can be part of the FFA syndrome, both well described in the literature.

Patient’s perspective.

The disease is extremely incapacitating, since it affects mainly my face and hair. I am ashamed of my rough skin, and everyone always asks me what is going on. It is also very frustrating how treatment has not improved the aspect of facial papules, even though it apparently prevents me from getting worse. I cannot wait to have my previous hair and skin back.

Learning points.

• Complete physical examination, including dermoscopy and trichoscopy, is critical to improve the diagnostic assertiveness.

• Histology is a helpful tool to confirm or rule out differential diagnosis in dermatology.

• Re-examine the patient’s signs and symptoms to avoid misdiagnosis.

Ethics statements

Patient consent for publication

Obtained.