Red face revisited disorders of hair growth and the pilosebaceous unit Marcia Ramos-e-Silva MD, PhD, Rodrigo Pirmez MD PII: DOI: Reference:

S0738-081X(14)00052-2 doi: 10.1016/j.clindermatol.2014.02.018 CID 6835

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Clinics in Dermatology

Please cite this article as: Ramos-e-Silva Marcia, Pirmez Rodrigo, Red face revisited disorders of hair growth and the pilosebaceous unit, Clinics in Dermatology (2014), doi: 10.1016/j.clindermatol.2014.02.018

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Marcia Ramos-e-Silva, MD, PhD Rodrigo Pirmez, MD

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RED FACE REVISITED DISORDERS OF HAIR GROWTH AND THE PILOSEBACEOUS UNIT

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1. Associate Professor and Chair – Sector of Dermatology and Supervisor of the Oral Dermatology Out-Patient Clinic – Sector of Dermatology and Post-Graduation Course – HUCFF-UFRJ and School of Medicine, Federal University of Rio de Janeiro, Brazil 2. Resident – Sector of Dermatology and Post-Graduation Course – HUCFF-UFRJ and School of Medicine, Federal University of Rio de Janeiro, Brazil From the Sector of Dermatology and Post-Graduation Course, HUCFF-UFRJ, and School of Medicine, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.

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Word count: 6759 Abstract word count: 73 Figures: 24 References: 82

The authors have no conflict of interest

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Correspondence to: Prof. Marcia Ramos-e-Silva Rua Dona Mariana 143 / C-32 Botafogo 22280-020 Rio de Janeiro Brazil [email protected]

ABSTRACT: The authors review the recent literature on the diseases of the hair and pilosebaceous unit that may cause a red face. We will discuss the epidemiology, clinics, pathogenesis, and therapy of lichen planopilaris with its variants, discoid lupus erythematosus, folliculitis decalvans, dissecting folliculitis, acne keloidalis nuchae, pseudofolliculitis barbae, tinea capitis, tinea barbae, folliculitis of diverse etiologies and inflammatory follicular keratotic syndromes, ulerythema ophryogenes, atrophoderma vermiculatum, keratosis follicularis spinulosa decalvans, and folliculitis spinulosa decalvans. KEY WORDS: Lichen planopilaris; frontal fibrosing alopecia; Lassueur Graham-Little Piccardi syndrome; folliculitis decalvans; discoid lupus erythematosus; dissecting folliculitis; acne keloidalis nuchae; pseudofolliculitis barbae; tinea capitis; tinea barbae, folliculitis; keratosis pilaris atrophicans; erythromelanosis follicularis faciei et colli; ulerythema ophryogenes; atrophoderma vermiculatum; keratosis follicularis spinulosa decalvans; folliculitis spinulosa decalvans.

ACCEPTED MANUSCRIPT INTRODUCTION There are several illnesses that may turn the face red and, among them, there are some disorders of hair and pilosebaceous follicle. We discuss the diseases

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related to the hair that may cause a red face and will present an update on their

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epidemiology, clinics, pathogenesis, and therapy. Most of them are causes of cicatricial alopecia of the scalp, but they can affect also the face, especially the area of the beard.

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The conditions presented are: lichen planopilaris with its variants, classic lichen planopilaris, Lassueur Graham-Little Piccardi syndrome, and frontal fibrosing

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alopecia, as well as fibrosing alopecia in a pattern distribution, which may be a fourth form of lichen planopilaris; discoid lupus erythematosus; folliculitis decalvans; dissecting folliculitis; acne keloidalis nuchae; pseudofolliculitis barbae; tinea capitis; tinea barbae; folliculitis of diverse etiologies and inflammatory follicular keratotic syndromes, including erythromelanosis follicularis faciei et colli; ulerythema

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ophryogenes; atrophoderma vermiculatum; keratosis follicularis spinulosa decalvans

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and folliculitis spinulosa decalvans.

LICHEN PLANOPILARIS

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Lichen planopilaris, a follicular form of lichen planus, is a rare inflammatory

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lymphocyte mediated disorder that selectively involves hair follicles. lichen planopilaris leads to follicular destruction and, consequently, cicatricial alopecia. Three forms of lichen planopilaris are described: classical lichen planopilaris, Lassueur Graham-Little Piccardi syndrome, and frontal fibrosing alopecia. Fibrosing alopecia in a pattern distribution could be added as a fourth variant. (1,2)

Classic lichen planopilaris Epidemiology Classic lichen planopilaris is largely seen in adults. Usually considered a rare disease, it is the most common cause of primary cicatricial alopecia.(1) It is more common in women and its onset usually occurs between ages of 40 and 60 years. Children are less usually affected.(1,3,4) Association with nonscalp lichen planus is

ACCEPTED MANUSCRIPT reported in 17-28% of cases at presentation and may occur during the course of disease in 50%.(3,4,5) Pathogenesis

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The cause of lichen planopilaris remains unknown. Based on similar

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histologic and direct immunofluorescence findings, it is suggested that lichen planopilaris results from the same pathologic process as lichen planus. An unknown antigenic trigger expressed by follicular keratinocytes seems to initiate the process.

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The inflammatory reaction occurs mainly around the bulge area and is mediated by T lymphocytes activated by Langerhans cells; which are increased in the dermis and

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epidermis. Certain drugs are known to trigger disease, but in most cases the cause is unknown.(1,3,5) Clinical Features

Lichen planopilaris frequently occurs in the vertex, but any region of the scalp can be involved. It may also be present at other sites such as the face, affecting

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eyebrows or beard or, more often, on arms, legs, axillary and pubic regions. (1) At the scalp, there may be solitary or multiple areas of baldness. The alopecic patches

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stretch centrifugally, sometimes in an irregular manner and can occasionally evolve to affect the entire scalp. Interestingly, unaffected hairs may be found within the lesion (Figure 1 and 2).(1,5) The most common symptoms are increased hair

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shedding, pruritus, scale and scalp tenderness.(4) Disease activity is usually seen in the hair-bearing margin and perceived as perifollicular erythema and scaling

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associated with anagen hair release upon execution of the pull test.(5) Diagnostic examination Trichoscopy allows better appreciation of the signs of disease activity as well as the perception that, in lichen planopilaris, perifollicular scaling tends to form tubular structures, considered highly characteristic of the disease.(6) Resolution of associated symptoms and the absence of visible inflammation do not, however, automatically imply that hair loss has been arrested. (7) Trichoscopy of the alopecic area may also reveal white dots indicating fibrotic tracts and pinpoint white dots, which are unspecific and represent openings of eccrine sweat ducts better visualized in patients of higher skin phototypes.(6,8) Blue-grey dots in a target pattern may be present, corresponding to pigment incontinence secondary to perifollicular interface dermatitis.(9) The presence of a pigmented network in a honeycomb pattern seen either in dark-skinned patients or in sun-exposed areas of the scalp is an important

ACCEPTED MANUSCRIPT feature to distinguish lichen planopilaris from discoid lupus erythematosus, since in the former the inflammatory process is restricted to the hair follicle, thus preserving the interfollicular architecture.(10) Elongated, parallel-oriented blood vessels at the

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margin of some active lichen planopilaris lesions have also been described.(6) (Figure

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3) Pathology

Histologic features consistent with early, active lichen planopilaris are

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follicular lymphocytic interface dermatitis, scattered dyskeratotic and necrotic keratinocytes in the basal layer, infundibular hyperkeratosis and hypergranulosis and

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atrophic or absent sebaceous glands. Pigmentary incontinence may be prominent. In late-stage lesions, inflammation can be minimal or absent and fibrous tracts take the place of destroyed hair follicles.(5) Loss of elastic tissue staining in a wedge-shaped configuration involving the upper third of the fibrous tract and associated destruction of the elastic sheath that marginates the fibrous tracts is a useful feature in late-stage

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lesions.(11) Direct immunofluorescence can be helpful in ambiguous cases, although it is not uncommonly negative. „„Shaggy‟‟ or „„patchy‟‟ deposition of fibrinogen and membrane zone. (3,5) Treatment

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clumped IgM or, less commonly, IgA and C3, is seen along the follicular basement

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Therapy is often not satisfactory.1,3) First-line treatment consists of intralesional or potent topical corticosteroids. As second-line therapy, oral

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corticosteroids, oral or topical ciclosporin, or oral tetracycline may be tried. Oral retinoids, antimalarials, thalidomide, griseofulvin, mycophenolate mofetil, low molecular weight heparin and excimer laser remain as third-line treatments.(7) The use of glitazone in the treatment of lichen planopilaris was reported to promote rapid resolution of symptoms and a decrease in inflammation on biopsy.(12)

Frontal fibrosing alopecia Epidemiology Frontal fibrosing alopecia typically affects postmenopausal women, but it can infrequently affect premenopausal women and, rarely, men.(1,5,13) Found in black or white populations, it seems to be less frequent in Asiatic people.(1) Pathogenesis

ACCEPTED MANUSCRIPT Frontal fibrosing alopecia is considered a variant of lichen planopilaris based on similar histopathology and coexistence of classic lichen planopilaris or lichen planus. The cause is uncertain, although an androgen related process might be

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speculated, given the strong association with postmenopausal status.( 5,14)

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Clinical Features

A progressive frontal or frontotemporal symmetric band of alopecia is the common presentation (Figure 4).(14) Sparing of isolated hairs within the alopecic band

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can be noted.(13) The skin at the recession area is smooth and shiny, contrasting with the rest of the facial skin and indicating where the former hairline used to be. At the

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new hairline, perifollicular erythema and mild scaling may be seen. Frontal fibrosing alopecia is usually asymptomatic, but mild pruritus might be a complaint. Lateral or complete eyebrow loss is a common feature and a diagnostic hint.(13,15) Nonscarring and noninflammatory symmetric axillary and extremity hair loss is not an uncommon finding.(5,13)

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Donati et al. described facial papules as an associated sign corresponding to vellus follicle involvement (Figure 5).(16) Association between frontal fibrosing

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alopecia and lichen planus pigmentosus in 24 patients has been recently reported.(17) Frontal fibrosing alopecia is usually insidious, but can be self-limited or rapidly

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progressive.(15)

Diagnostic examination

Trichoscopy shows the perifollicular erythema and scaling associated with a

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lack of follicular ostia and an ivory-white background (Figure 6).(18) Trichoscopic examination may also help in the differentiation from traction alopecia, alopecia areata in sisaipho pattern and androgenetic alopecia.(15) Pathology Routine histopathology, direct immunofluorescence and immunohistochemical studies resemble classic lichen planopilaris, but vellus-like and intermediate hairs appear to be more commonly affected than terminal hairs.(14,15) Treatment There is no proven effective treatment for frontal fibrosing alopecia.(1,5,14) Moreover, the absence of inflammatory signs does not necessarily mean halting disease progression.(13) The protocol for classic lichen planopilaris may be followed.(7) Additionally, the use of finasteride has been reported.(19) According to

ACCEPTED MANUSCRIPT some authors, hair transplants can be proposed when there is no progression of the disease for at least 1 year.(1)

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Lassueur Graham-Little Piccardi Syndrome

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Epidemiology

Lassueur Graham-Little Piccardi syndrome is a rare disease with most of the cases being described in female adults between the ages of 30 and 60 years-old.(1)

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Pathogenesis

Lassueur Graham-Little Piccardi syndrome is classically considered a variant

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of lichen planopilaris, but some classify it as type of keratosis pilaris atrophicans, while others consider it to be a distinct nosologic entity.(5,14) Clinical Features

Lassueur Graham-Little Piccardi syndrome consists of the triad of progressive scarring scalp alopecia, nonscarring loss of pubic and axillary hair and

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disseminated follicular papules with spinous scales. These features may appear simultaneously, but scalp alopecia often precedes the follicular eruption.(1) Pruritus

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may be present. Active scalp disease can resemble either classic lichen planopilaris or assume the configuration of erythematous, scaly, patchy alopecia. Progression of

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hair loss is variable. The face and eyebrows are infrequently involved.(14) Diagnostic examination

Trichoscopy of the scalp is comparable to classic lichen planopilaris. In the

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axillae and pubic area hairs become thinned and eventually disappear.(18) Pathology

Histopathological features of both classic lichen planopilaris and keratosis pilaris atrophicans have been described.(5,14) Treatment Treatments are similar to those used for classical lichen planopilaris

(1,7).

Follicular keratosis of the trunk and extremities may respond to topical tretinoin.(14 ) Fibrosing Alopecia in a Pattern Distribution Described in 2000, this new form of cicatricial alopecia is not yet recognized by all authors as an authentic entity.(1,2) Patients with androgenetic alopecia present with acute deterioration of their hair loss. Cicatricial alopecia is limited to the area of androgenetic hair loss. Perifollicular erythema is universally seen, often with follicular

ACCEPTED MANUSCRIPT keratosis. Pain and pruritus might be associated.(2,14) Pathology in early disease reveals hair follicle miniaturization and a lichenoid inflammatory infiltrate around the upper follicle. Advanced lesions correlate with perifollicular lamellar fibrosis and

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completely fibrotic follicular tracts.(2) Four patients of the original cohort responded to

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finasteride.(2)

DISCOID LUPUS ERYTHEMATOSUS

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Epidemiology

Discoid lupus erythematosus typically starts between the ages of 20 and 40

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years and has a female predominance (5 20). Less than 5% of adults with discoid lupus erythematosus will develop systemic lupus erythematosus. Such progression, however, seems to be more common in children (26%) Pathogenesis

(20,21).

Manifestation of discoid lupus erythematosus results from an intricate

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interaction between genetic, enviromental and host factors. In predisposed patients, ultraviolet radiation might induce apoptosis of keratinocytes and immunosuppression followed by an autoreactive inflammatory response leading to epithelial destruction.

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Clinical Features

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Deficient clearance of apoptotic cells contributes to perpetuation of disease.(5,14)

Discoid lupus erythematosus is the only form of chronic cutaneous lupus capable of causing cicatricial alopecia.(5) (Figure 7 and 8) Commonly, scalp is the first

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site of involvement and the vertex the most frequently affected site.(20,22) Patients may present with single or multiple lesions or even with a “footprints in the snow” appearance, resembling what has been considered the classic lesion of pseudopelade of Brocq.(22) The most common symptoms are increased hair shedding, pruritus and scalp tenderness.(4) Patients may also complain of stinging and burning.(5) Early lesion is characterized as a well-defined erythematous papule or plaque. The lesions spread centrifugally, thicken, loose hair and develop adherent scale and follicular plugging. In contrast to many other cicatricial alopecias, these clinical findings are typically seen in the center of the alopecic patch rather than around the rim of hair. In late stage disease, atrophy, telangiectasia and dyschromia become prominent features. Recurrences often present in the center of former lesions.(5,20) Diagnostic examination

ACCEPTED MANUSCRIPT A positive pull test with anagen hairs and prominent follicular keratosis reflects disease activity.(4) Trichoscopic clues in the diagnosis of discoid lupus erythematosus include follicular red dots that correspond to dilated infundibula

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surrounded by dilated vessels and extravasated erythrocytes. They represent a good

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prognosis and chance of hair regrowth.(23) Other hints for the differentiation of discoid lupus erythematosus from other causes of alopecia are blue-grey dots in a speckledpattern representative of pigment incontinence secondary to interface dermatitis; and

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also, the absence of a pigmented network, for discoid lupus erythematosus tends to destroy the interfollicular architecture.(9) Other common trichoscopic features of

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discoid lupus erythematosus of the scalp are large yellow dots, thick arborizing vessels and, in long-stand lesions, the presence of milky-red areas without follicular orifices.(6,18) (Figure 9 and 10) Pathology

Histopathological features of acute discoid lupus erythematosus include

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interface dermatitis with basal vacuolization, dyskeratotic keratinocytes, cytoid bodies and a predominantly lymphocytic inflammatory infiltrate around superficial and

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deeper vasculature as well as around adnexal structures. As the disease progresses, ortho-hyperkertosis with areas of parakeratosis and follicular plugging may be noted.

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There is epidermal atrophy, basement membrane zone thickening and pigment incontinence. Concentric lamellar fibrosis is an end-stage finding. At this stage, elastic tissue staining reveals diffuse dermal scarring and no evident follicular elastic

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sheath.(11,14) Mucin deposition is a further clue for the diagnosis. DIF reveals a granular or linear pattern of IgG, IgM and C3 deposition along the dermal-epidermal junction.(11) Treatment Unlike most types of cicatricial alopecia, hair regrowth can be expected if the lesion is treated in its early stage. As discoid lupus erythematosus commonly worsens with sun-exposure, photoprotection should be advised. In limited, active disease potent topical steroids or intralesional triamcinolone acetonide or both are first line therapy.(5,7) Second-line or adjunctive topical agents include calcineurin inhibitors, retinoids and imiquimod with mixed outcomes. (7,14) Patients with rapidly progressive, disfiguring or refractory disease may need systemic treatment. In such cases, antimalarials are first line therapy. Improvement is seen in 4-8 weeks. During this period, a short course of systemic steroids may be needed.(5) Oral retinoids have

ACCEPTED MANUSCRIPT the same benefit of antimalarials, however with greater side-effects.(24) Several additional therapies for discoid lupus erythematosus have been proposed, including thalidomide,

methotrexate,

dapsone,

clofazimine,

mycophenolate

mofetil,

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azathioprine, among many others.(7)

FOLLICULITIS DECALVANS Epidemiology

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Folliculitis decalvans patients represent 11% of all primary cicatricial alopecia cases, being the most common type of neutrophilic scarring alopecia. FD is a Americans and male patients.(4,25). Pathogenesis

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disease of young and middle aged-adults and seems to be more common in African-

The pathogenesis of folliculitis decalvans remains poorly understood. Staphylococcus aureus is frequently cultured from lesions and has been implicated

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as a causative agent.(4,25,26) It has been suggested that superantigens and an exaggerated immune response might play a role in the pathogenesis of the disease. confirmed.(25,26)

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Clinical Features

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However, no disturbance in systemic or local immune response has been

Early lesions of folliculitis decalvans are erythematous follicular papules that are mainly found at the vertex and occipital areas of the scalp. These lesions

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progress to round to irregularly shaped alopecic patches that characteristically present with pustules, erosions and yellow and hemorrhagic crusts at the advancing margins.(5,26,27) Pain, itching and burning sensation are frequent complaints.(27) Eventually, the center of the lesions becomes atrophic with flesh colored or ivorywhite areas.(5) The development of hair tufts consisting of multiple hairs (5-20) emerging from one single dilated follicular orifice is characteristic, but not pathognomonic. If compression is applied to the perifollicular area, discharge of purulent material may be seen.(27) Hair tufts usually occur at the periphery of the lesion and persist even when inflammation has cleared.(25) (Figure 11) Diagnostic examination Trichoscopy helps to better visualize the clinical features already described. Tufted hairs surrounded by perifollicular hyperplasia arranged in a starburst pattern have been described as a specific trichoscopic feature (Figure 12).(6)

ACCEPTED MANUSCRIPT Pathology In early stage lesions, histologic examination reveals acneiform follicular dilation with an intrafollicular and perifollicular neutrophilic inflammatory infiltrate

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involving the upper and middle portions of the hair follicle. With advancing disease,

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lymphocytes, histiocytes and, particularly, plasma cells are also observed. Late-stage lesions are characterized by follicular and adventitial dermal fibrosis. PAS stain to exclude a fungal infection should be performed.(11,26)

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Treatment

Material from intact pustules should be collected for bacterial culture aiming

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antibiotic selection. Since tinea capitis may have a similar presentation, fungal culture should also be considered.(28) Successful reports of the use of the association of oral rifampicin and clindamycin seem to have changed the management of this seemingly intractable disease. A 10-week cycle is advocated. Some patients may need a second or third 10-week course, but most of the patients in the original report noted a

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distinct improvement without relapse. Association with another antibacterial agent is always advisable, because rapid emergence of bacterial resistance will ensue if rifampicin is used alone. Other possible drugs to be used in combination are

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doxycycline, ciprofloxacin or clarithromycin.(25,29) Traditionally, the use of oral

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antistaphylococcal antibiotics in association or not with topical antibiotics have been used and reported to improve folliculitis decalvans; however, with frequent relapse of the disease after discontinuation.(7) Topical, intralesional or even oral corticosteroids

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can be considered in order to help reduce inflammation and symptoms, especially in very symptomatic and rapidly progressing disease.(29) Numerous other treatments have been reported with little evidence, limited effectiveness or contradictory results. DISSECTING FOLLICULITIS Epidemiology Dissecting folliculitis is reported to occur mainly in young black men and represented approximately 1% of all cases of cicatricial alopecia in one study.(26) Pathogenesis Dissecting folliculitis is part of the follicular oclusion tetrad, together with hidradenitis suppurativa, acne conglobata and pilonidal cysts. These disorders are characterized by follicular hyperkeratosis resulting in follicular occlusion, secondary bacterial infection and follicular rupture. Foreign-body reaction and scarring

ACCEPTED MANUSCRIPT ensues.(7) Clinical Features Early lesions of dissecting folliculitis typically occur at the vertex and occipital

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regions of the scalp and consist of follicular pustules.(30) These lesions rapidly

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develop into painful erythematous nodules and abscesses with interconnecting sinus tracts. (Figure 13) The pull test is often positive in the areas of inflammation. The nodules might suppurate spontaneously evolving into atrophic scarring or keloidal (5,30)

Pressure on one nodule can result in expression of seropurulent

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alopecia.

exudate directly or from an adjacent interconnected nodule

(31).

Occipital

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lymphadenopathy and an elevated serum erythrocyte sedimentation rate are often observed. The disease usually follows and chronic and relapsing course, but may be self-limited.(14) Diagnostic examination

Trichoscopy features of dissecting folliculitis already described include yellow

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dots, red dots, empty follicular openings and black dots (Figure 14).(32) End-stage disease is characterized by unspecific findings such as confluent ivory-white areas

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lacking follicles.(18) Pathology

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Acneiform distention of the follicular infundibula with keratotic plugging and intra- and perifollicular neutrophilic infiltrate is an early finding in dissecting folliculitis. Follicular rupture leads to subcutaneous abscesses formation. There is late

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recruitment of lymphocytes, plasma cells and foreign body giant cells. The hallmark of the disease is formation of sinus tracts lined by stratified squamous epithelium. Late-stage disease is characterized by the substitution of follicular units by scar tissue, diffuse dermal fibrosis and persistence of sebaceous glands.(11,14) Treatment Successful case reports underlie the recommendations to consider oral isotretinoin as first-line treatment for dissecting folliculitis.(7,33) The suggested dosage is 1 mg/kg/d for 4 months, followed by 0.75 mg/kg/d for a further 6 months. Association with periodic intralesional triamcinolone acetonide injections has been reported with long-lasting remission.(34) In severe cases, oral corticosteroids may be considered.(26) An extensive review on the management of primary cicatricial alopecia considered oral antibiotics, topical antibiotics plus topical retinoids, oral zinc, colchicine, dapsone, among other modalities reported, as second or third line

ACCEPTED MANUSCRIPT strategies for the treatment of dissecting folliculitis.(7) The use of anti-TNF alpha agents has been reported in refractory cases with a dramatic treatment

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response.(35,36)

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ACNE KELOIDALIS NUCHAE Epidemiology

Acne keloidalis nuchae predominantly affects young black males.(5)

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representing 1.3% of all skin conditions seen in a dermatologic clinic of an African country.(37) There are case reports of drug-induced acne keloidalis nuchae in patients carbamazepine, in one case.(39) Pathogenesis

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using ciclosporin following organ transplantation;(38) and diphenylhydantoin and

The pathogenesis of acne keloidalis nuchae is poorly understood. Acne keloidalis is a misnomer, since the disease is not a variant of acne vulgaris, nor is

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keloidal in nature.(40) Sperling et al.(41) proposed that antigens of the follicular epithelium or within the follicular canal attract inflammatory cells at the level of the

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isthmus. The perifollicular inflammation would be responsible for weakening the follicular wall, eventually causing the hair shaft to migrate through the wall into the

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dermis, provoking an intense inflammatory reaction and epithelial destruction. In some individuals, hair shaft fragments and degenerating epithelial components incite the hypertrophic scarring. The sebaceous gland would be either an initial target or an

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“innocent bystander” destroyed early in the inflammatory process. Another study suggested an association with increased fasting blood testosterone levels. sporadic occurrence of drug-induced acne keloidalis nuchae

(38,39)

(37)

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might further shed

light on the pathogenesis of the disease. Clinical Features Acne keloidalis nuchae (Figure 15) begins with small, flesh-colored, pink or reddish brown dome-shaped papules on the nape of the neck or occipital scalp. Pustules may also be present. Initially, hairs may be seen exiting the papules. The lesions are often pruritic and sometimes painful.(14,40) Later in the disease, more papules may appear and some coalesce to form large hairless keloid-like plaques.(40) Pathology Characteristic findings include neutrophilic or lymphoplasmocytic perifollicular infiltrate at the isthmus, lower infundibulum and around sebaceous glands, which are

ACCEPTED MANUSCRIPT lost early in the disease; a thinned outer root sheath with reparative concentric lamellar fibroplasia; hair shaft granulomas and extensive dermal hypertrophic scarring.(11,41)

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Treatment

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Acne keloidalis is a chronic condition, unlikely to remit without treatment.(5) Mild disease may be treated with potent topical steroids alone or in association with topical antibiotics.(40) Treatment of papular lesions includes intralesional triamcinolone

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acetonide (10-40mg/ml every 4 weeks). Oral antibiotics (eg, tetracylines) may help.(25,40) In advanced disease, surgical excision, which can be done in stages, is an

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option. Both excision with primary closure or secondary intention healing with good results have been reported. However, there is a risk of partial recurrence. (42,43) PSEUDOFOLLICULITIS BARBAE Epidemiology

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Pseudofolliculitis barbae is primarily seen in the beard area of individuals with tightly curled hairs who shave; mainly men of African American and Hispanic

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origins;(40,44) pseudofolliculitis barbae also occurs in women from tweezing or plucking facial hair. Other areas of hair removal, such as axilla and groin, may also be

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involved.(45) Males are mostly affected from 14 to 25 years old and women in their perimenopausal period, due to changes in hormone levels. Hirsute women may be affected at an earlier age.(44)

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Pathogenesis

The direction of hair growth in affected individuals plays an important role in the pathogenesis of pseudofolliculitis barbae. After shaving, the hair tip becomes sharp. As the hair grows, because of its natural tendency to curve, it ends-up piercing the adjacent skin surface and re-entering the epidermis (extrafollicular penetration), 1 to 2 mm away from the hair follicle.(44) Stretching the skin during shaving may also favor transfollicular penetration. After releasing the skin, the shaved hair retracts below the skin surface. It then grows within the hair follicle, penetrating the follicle wall into the dermis. In both situations, the presence of the hair shaft will lead to a foreign body reaction within the dermis.(40,44) A study demonstrated that a singlenucleotide polymorphism gives rise to a disruptive Ala12Thr substitution in the 1A ahelical segment of the companion layer-specific keratin K6hf of the hair follicle, being

ACCEPTED MANUSCRIPT partially accountable for the phenotypic expression and denoting a genetic risk factor for PFB.(46) Clinical Features

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The characteristic lesions of pseudofolliculitis barbae are erythematous, skin-

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colored or hyperpigmented papules ranging from 2mm to 4 mm in a follicular distribution. The most common sites of involvement are the neck, in men, and the chin, in women (Figure 16).(44) Often, a hair can be seen within the papule.(47)

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Pseudofolliculitis barbae severity varies from mild (fewer than a dozen lesions) to severe disease (more than 100 lesions).(40) Pustules or papulopustules are thought to

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represent secondary infection. Post-inflammatory hyperpigmentation and keloids may result from chronic inflammation. (44) Diagnostic examination

Dermoscopy of individual papules may reveal the presence of U-shaped ingrowing hairs.(47) (Figure 17)

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Pathology

Histopathology shows invagination of the epidermis produced by the hair tip

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and foreign body giant cell reaction within the dermis. Other features are abscess formation within the pseudofollicle, naked shafts surrounded by acute and chronic Treatment

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inflamation and fibrosis.(44,45)

Patient education is the first step for the treatment of pseudofolliculitis

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barbae. Avoiding shaving and other techniques of hair removal such as waxing, tweezing and electrolysis may resolve the situation for most of the patients. The embedded hairs pull themselves out of the epidermis by natural tension in 3 to 6 weeks when they have grown 1 cm or more in length. Trimming the hair with scissors or electric clippers to a minimum length of 1 cm is another option. However, this might not be acceptable for some patients. There are special shaving apparatus destined for patients with pseudofolliculitis barbae that have shown benefit. General orientation for patients who must shave, such as moistening the skin with warm water, not pulling the skin taut while shaving and shaving with the grain, are helpful. Another possibility is the use of chemical depilatories.(40,44,48) Among the medical treatments there are topical steroids, tretinoin,(49) association of benzoyl peroxide with clindamycin,(50) glycolic acid

(51)

and eflornithine hydrochloride.(44) However, laser hair

removal has revolutionized the way that these patients can be treated and should be

ACCEPTED MANUSCRIPT seriously considered. Laser epilation can be accomplished using pulsed alexandrite (755 nm), diode (800–810 nm), neodymium: yttrium aluminum garnet (Nd:YAG; 1064

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nm), among other systems available on the market.(48)

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TINEA CAPITIS Epidemiology

Tinea capitis is a dermatophytosis of the scalp and associated hair. It is the

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most common fungal infection in children, being uncommon in adults. Pathogenic organisms vary from country to country. In a Brazilian study, Microsporum canis

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(70,5%) and Trichophytum tonsurans (23,2%) represented the most common agents, followed by T. mentagrophytes (3.6%), M. gypseum (1.8%) and T. rubrum (0.9%).(52) Pathogenesis

There are three patterns of hair infection: endothrix, ectothrix and favus (endothrix favosa). Endothrix is characterized by the presence of arthoconidia within

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the hair shaft, replacing keratin inside the hair and leaving the cortex intact. In ectothrix infections, arthroconidia attaches to the surface of the hair shaft, which

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eventually leads to cuticle destruction. Typical pathogens involved in ectothrix are M. canis and M. audouinii as well as T. mentagrophytes var. granulosum. Endothrix can

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result from infection by pathogens such as T. tonsurans or T. violaceum.(53) Trichophyton schoenleinii is the most frequently identified causative organism in cases of favus. In patients with tinea capitis favosa, infected hairs are characterized

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by the presence of hyphae and air bubbles within the hair shaft.(54) According to their preferential habitat, the dermatophytes may be classified as anthropophilic, zoophilic or geophilic; influencing clinical features in human infections. However, identification of the pathogen is not possible based solely on clinical presentation.( 53) Clinical Features The presentation of tinea capitis may vary from subtle asymptomatic scaling to inflammatory suppurative forms, depending on its etiology and host immune factors.(55) (Figure 18,19 and 20) Ectothrix organisms commonly produce a seborrheic form with scaling and minimal inflammation. Often, there is no perceivable loss of hair. However, these lesions may also appear as circumscribed gray patches of alopecia, due to the breaking off of hairs. The black-dot type of tinea capitis is produced by endothrix organisms such as T. tonsurans and T. violaceum that cause breakage of the hair shaft at the level of the scalp, leaving an appearance of grouped

ACCEPTED MANUSCRIPT black dots with variable degrees of inflammation. Inflammatory lesions of tinea capitis usually occur with zoophilic and geophilic organisms and range from pustular folliculitis to kerion. (53,56) Kerion (Figure

and

follicular

pustules,

often

associated

with

pain

and

cervical

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hairs

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19) is characterized by single or multiple inflammatory masses studded with broken lymphadenopathy. Such lesions often resolve with scarring alopecia. (53,56) Tinea capitis favosa is a chronic inflammatory dermatophyte infection of the

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scalp and, less commonly, glabrous skin and nails. T. schoenleinii is the most common cause of human favus. In early disease, there is only erythema, mainly

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around hair follicles. With disease progression, there is formation of concave, cupshaped yellow crusts, known as scutula, along with hair loss. In advanced disease, large areas of the scalp are involved with marked hair loss, atrophy and scarring. Commonly, there is development of new scutula at the periphery of plaques.(54) Diagnostic examination

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Wood light examination may reveal yellowish-green fluorescence confirming a diagnosis of Microsporum infection. In tinea capitis favosa, a faint blue

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fluorescence may be present.(54)

The presence of comma hairs and corkscrew hairs (Figure 20) seen under trichoscopy is another helpful tool for the clinical diagnosis of tinea capitis, regardless

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of the etiological agent.(57)

An interesting clinical clue recently described for the clinical suspicion of

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tinea capitis is the „ear sign‟, which means the presence of erythematous papules, scaling or well to ill-defined plaques over helix, antihelix and retroauricular region (away from retroauricular fold).(58) Pathology Clinical diagnosis of tinea capitis is confirmed by unstained specimens and fungal culture. Histologic analysis with fungal staining may be useful in culturenegative patients who are already undergoing treatment, revealing hyphae around and within the hair follicle. In the dermis, there is mixed perifollicular inflammatory infiltrate. Follicular disruption leads to foreign-body giant cell reaction.(53,56) Lesions with severe inflammation, such as kerion, are characterized by an inflammatory response with neutrophilic or granulomatous infiltrates, or both, that in later stages result in a fibrotic scar.(59) Treatment

ACCEPTED MANUSCRIPT Oral griseofulvin is still considered gold standard therapy for tinea capitis.(55) However, a meta-analysis of randomized controlled trials comparing griseofulvin and terbinafine did not show a significant difference in the overall efficacy of the two

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drugs, but specific efficacy differences were observed based on the infectious

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species. For tinea capitis caused by Microsporum spp., griseofulvin is superior, whereas terbinafine is superior for Trichophyton spp. Infection.(60) The goal of treatment is mycological cure. Treatment progress should be monitored every

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fourteen days starting with the fourth week of treatment. Oral fluconazole and itraconazole are other possible options described.(53) Adjuvant therapy with

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shampoos such as selenium sulfide 1-2,5%, zinc pyrithione 1-2%, povidone iodine 2,5% or ketoconazole 2% are commonly used. Although there is no consistent evidence for different cure rates, the association of oral prednisone 1-2mg/kg/day for the first week of therapy in severe inflammatory tinea capitis is beneficial in relieving

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pain and swelling.(56) TINEA BARBAE

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Epidemiology

Tinea barbae is a rare dermatophytic infection of the bearded areas of the

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face and neck with invasion of coarse hairs. Tinea barbae is by definition seen only in males. When present at the chin and upper lip in female and children, it is considered tinea faciei. (61,62)

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Pathogenesis

Although there are only few studies about tinea barbae, the available clinical data points to a pathogenesis similar to tinea capitis.(62) In the past, the main way of transmission relied on the fact that barbers did not use disposable razors. However, better hygiene has reduced its incidence, nowadays. Tinea barbae is now more often transmitted by direct contact with animals, thus more commonly seen in rural setting among farmers or ranchers.(56) Some authors have reported TB occurring after autoinoculation from fingernails or tinea pedis.(61,63) Clinical Features According to the clinical findings, tinea barbae may be classified into three different types: inflammatory, superficial and circinate. The inflammatory form of tinea barbae is usually caused by T. mentagrophytes or T. verrucosum and is comparable to kerion formation that may be seen in some cases of tinea capitis.(56) Lesions are

ACCEPTED MANUSCRIPT nodular with exsudate and crusts covering its surface. Hairs may be easily plucked. Regional lymphadenopathy, fever and malaise may be associated features. Scaring alopecia may ensue.(61) The superficial type is normally related to antropophilic

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organisms and resembles bacterial folliculitis, with mild background erythema and

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perifollicular papules and pustules. The circinate type is characterized by its active erythematous, vesiculopustular border and relative sparing of the hair, as seen in other parts of the body.(56) (Figure 21)

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Diagnostic examination

Wood light examination may reveal dull green fluorescence of the affected

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hairs in the rare cases of tinea barbae caused by Microsporum canis.(61) Pathology

The diagnosis of tinea barbae relies on mycologic investigation with direct microscopy and culture. Histological examination is destined for cases in which clinical diagnosis is not evident. It may show folliculitis and perifolliculitis with

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spongiosis and lymphocytic follicular infiltrates. Sometimes microabscesses are formed by neutrophils within follicular keratin. Fungal elements may be detected in

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stratum corneum, hair follicle and hair shaft.(61) Treatment

Like in tinea capitis, oral antifungal agents are required over a period of 4-6

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weeks. Combination with topical therapy may be used.(62,64) In severe inflammatory

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lesions, systemic steroids are helpful.(56) Late recurrences may occur.(62)

FOLLICULITIS AND INFLAMMATORY FOLLICULAR KERATOTIC SYNDROMES Folliculitis is characterized by the presence of inflammatory cells within the wall and ostia of the hair follicle, creating a follicular-based pustule arising on an erythematous base.(65) There are many causes of folliculitis, such as bacteria, viruses, fungi and parasites, as well as non-infectious causes; and classification is controversial. Here, we will mainly consider the causes in which „red face‟ is a clinical feature. A group of disorders characterized by abnormal follicular keratinization will also be addressed in this topic. Infectious Folliculitis Bacterial Folliculitis

ACCEPTED MANUSCRIPT Bacterial folliculitis includes superficial and deep infection of the hair follicle with Staphylococcus aureus being the most common causative agent. Superficial folliculitis

(osteofolliculitis)

usually

manifests

as

painful

erythematopapular

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perifollicular lesions on the upper lip of children or on the beard and other hairy

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areas, in adults.(65) When there is involvement of the entire follicle and surrounding tissue, a furuncle develops. A contiguous collection of inflamed and infected hair follicles is named carbuncle.(66) Bacterial sycosis is a subacute or chronic

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staphylococcal infection involving the entire follicle that occurs in the beard area of males after puberty and is characterized by an inflammatory plaque studded with

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pustules, accompanied by a burning sensation. The chronic severe form (lupoid sycosis) may result in scarring.(67) Predisposing factors to folliculitis include occlusion, maceration, hyperhydration and application of topical steroids, among others.(67) Gram-negative bacterial folliculitis due to Klebsiella, Enterobacter and Proteus spp. is usually seen in patients with acne vulgaris receiving long-term antibiotic therapy. It is

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characterized by pustules in the facial T-zone and in the perinasal area.(68) Syphilitic folliculitis is a rare form of secondary syphilis, which may appear in the frontal hairline and nasogenian folds; its progression resulting in grouped linear or polycyclic

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arranged elements.(65) The diagnosis of bacterial folliculitis is usually made on clinical

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grounds. Gram stain and culture may help to identify the causative organism. Superficial bacterial folliculitis normally responds to topical antibacterials. When widespread or recurrent, systemic antibiotics should be considered. In cases of deep

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follicular infection, appropriate oral antibiotic based on culture results is mandatory. In severe Gram-negative BF, oral isotretinoin is an option. (68) Fungal Folliculitis

Fungal folliculitis is divided into three groups: dermatophytic, which has been already addressed, Pityrosporum and Candida folliculitis.(67) Pityrosporum folliculitis is mainly found on the back, upper trunk and shoulders, but does not typically involves the face; therefore it will not be discussed here. Folliculitis caused by Candida albicans is a rare condition predominantly limited to hair follicles of beard and moustache area in men, and less frequently to the scalp.(69) It is classically reported in individuals with intravenous heroin abuse, in whom painful papules, nodules and pustules are accompanied by systemic symptoms.(67) However, other risk factors such as orogenital sexual contact have been raised, due to observation of this

ACCEPTED MANUSCRIPT disorder in healthy individuals.(69) Candida folliculitis must be treated with oral itraconazole 200mg/day.(67) Viral Folliculitis

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Folliculitis due to herpes simplex virus (HSV) and molluscum contagiosum

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are rare entities and should be considered a sign of immunosuppression, especially in cases of folliculitis due to molluscum contagiosum.(70) Herpetic folliculitis is usually seen in men with a history of recurrent HSV infection on the face who use blade

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razors. Folliculitis due to HSV manifests as multiple pruritic erythematous papules or papulovesicles with crusts or umbilicated vesicles on the face and should be treated

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with oral antiviral therapy.(67,68). The clinical presentation of molluscum contagiosum folliculitis consists of several pruritic whitish or erythematous papules over the beard area. Investigation for immune deficiency is mandatory. Treatment can be achieved with curettage of the lesions, among other modalities.(67,70) Parasitic Folliculitis

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Demodex is usually a saprophyte on humans, feeding from gland secretions, and causing no symptoms. However, D. folliculorum has also been implicated in many cutaneous pathologies, including rosacea, granulomatous perioral dermatitis,

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blepharytis, as well as folliculitis(67,71) Demodex follicullitis is characterized by erythematous follicular papules on the face, often with a background of diffuse

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erythema. Skin scrapings reveal numerous demodex mites.(68) Since Demodex is a saprophyte in the human skin, a causal relationship with those disorders has always

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been controversial. Treatment strategies include topical 5% permethrin cream, oral ivermectin or itraconazole.(67,68) Non-Infectious Folliculitis Eosinophilic pustular folliculitis Eosinophilic pustular folliculitis was first described by Ofuji, in 1970. The pathogenesis, however, remains uncertain. There are three major forms of the disease: classic eosinophilic pustular folliculitis or Ofuji disease, HIV-associated eosinophilic pustular folliculitis, and eosinophilic pustular folliculitis of infancy.(67) Ofuji disease is more common in men around 30 years of age and is characterized by recurrent episodes of intensely pruritic grouped erythematous follicular papulopustules, accompanied by leukocytosis and eosinophilia. Lesions occur mainly in seborrheic areas, such as the face and back, however palms and

ACCEPTED MANUSCRIPT soles may be affected in some cases. Additionally, there are erythematous patches and plaques superimposed by pustules, with central clearing and centrifugal expansion, producing lesions with annular and serpiginous aspects. Lesions last

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about 7-10 days.(65,68)

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In contrast, HIV-associated eosinophilic pustular folliculitis is usually chronic and presents as an erythematous follicular papular eruption of the face, scalp and upper trunk. However, there may also be non-follicular papules, urticariform lesions

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or even generalized rash. Pruritus is intense and often severely excoriated. Palms and soles are not affected.(67,68)

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In eosinophilic pustular folliculitis of infancy, all patients show lesions on the scalp, tissue eosinophilia, and recurrent outbreaks. Other inconstant but highly indicative findings are pruritus and blood eosinophilia. Eosinophilic pustular folliculitis of infancy occurs essentially in children less than 1 year of age and is more common in male patients.(72)

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Histologic features are similar among the three variants of the disease and include spongiosis with exocytosis of lymphocytes and eosinophils into the follicular

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epitelium. Micropustular aggregation ensues, followed by the hallmark of eosinophilic pustular folliculitis, infundibular eosinophilic pustules. The pathogenesis of the disease remains unknown.(68) The first line of therapy in all eosinophilic pustular

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folliculitis variants is topical steroids and the gold standard UVB phototherapy.(73)

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Inflammatory follicular keratotic syndromes Keratosis pilaris is a common condition represented by excessive follicular keratinization leading to follicular plugging with varying degrees of surrounding erythema. It may occur alone or accompanying different pathologic processes, characterizing different syndromes.(74) In erythromelanosis follicularis faciei et colli, well-demarcated erythema involving the cheeks and lateral aspects of the neck, hyperpigmentation, and follicular papules with onset generally during childhood are the distinguishing features. Some patients may also have associated keratosis pilaris lesions on the trunk.(75) The pathogenesis is unknown and histologic findings are unspecific, including follicular hyperkeratosis, dilation of upper dermal vessels, perivascular inflammatory infiltrate and increased basal layer pigmentation.(74,76) There is no

ACCEPTED MANUSCRIPT proven effective therapy and treatments tried include various topical keratolytic agents, chemical peels, hydroquinone, oral isotretinoin and laser treatments.(68,75) Keratosis

pilaris

atrophicans

represent

a

group

of

rare

diseases

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characterized by keratosis pilaris on the extensor surfaces, inflammation, and

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atrophic scarring. Keratosis pilaris atrophicans spectrum includes ulerythema ophryogenes, atrophoderma vermiculatum and keratosis follicularis spinulosa decalvans. A variant of keratosis follicularis spinulosa decalvans with distinct clinical

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features has been named folliculitis spinulosa decalvans.(77)

Most cases of ulerythema ophryogenes, also known as keratosis pilaris

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atrophicans faciei, follow an autosomal dominant inheritance pattern, with incomplete penetrance.(78) Onset of the disease generally occurs a few months after birth, typically presenting with erythema and small keratotic follicular papules at the lateral third of the eyebrows. It may progressively spread to the forehead and cheeks. Superficial atrophy and alopecia develop during later stages. Disease progression

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usually ceases after puberty.(77,78)

Atrophoderma vermiculatum usually begins in childhood, between 5 and 12

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years old, with erythema and follicular keratotic papules as well as milia on the cheeks. atrophoderma vermiculatum slowly progresses to atrophic pits, giving the

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cheeks a reticulate, honeycomb, or worm-eaten appearance.(78) Keratosis follicularis is a common associated feature. Rarely, upper lip, helices, ear lobes and limbs may be affected. Alopecia is not a feature of the disease. atrophoderma vermiculatum is

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usually sporadic, but autosomal dominant pattern of inheritance has been reported.(78,79)

Keratosis follicularis spinulosa decalvans presents with diffuse Keratosis pilaris, scarring alopecia of the scalp and associated photophobia, facial erythema, and palmoplantar keratoderma.

Disease onset occurs during infancy. Although

initially described as a sex-linked disorder, several different inheritance patterns have been

observed.(81) Similar

to

ulerythema

ophryogenes

and

atrophoderma

vermiculatum, it tends to remit after puberty. Folliculitis spinulosa decalvans, on the other hand, is characterized by persisting pustule formation and more severe inflammation, associated to typical keratotic papules of the scalp and scarring alopecia.(82) It appears to have an autosomal dominant pattern of inheritance and tendency to exacerbate after puberty.(74).

ACCEPTED MANUSCRIPT Treatment of these disorders is challenging and there are only case reports in the literature, where detailed information can be found.(77-81)

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CONCLUSION

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Red face is a common manifestation of many cutaneous and systemic diseases. Dermatologists should be aware of these illnesses in order to make an accurate diagnosis. Among them, the various diseases of the hair and pilosebaceous

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difficult. In some the therapy is often frustrating.

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unit are included and the differential diagnosis between them is frequently very

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35. Brandt HR, Malheiros AP, Teixeira MG, Machado MC. Perifolliculitis capitis abscedens et suffodiens successfully controlled with infliximab. Br J Dermatol. 2008;159:506-507. 36. Wollina U, Gemmeke A, Koch A. Dissecting cellulitis of the scalp responding to intravenous tumor necrosis factor-alpha antagonist. J Clin Aesthet Dermatol. 2012;5:36-39. 37. George AO, Akanji AO, Nduka EU, Olasode JB, Odusan O. Clinical, biochemical and morphologic features of acne keloidalis in a black population. Int J Dermatol. 1993;32:714-716. 38. Azurdia RM, Graham RM, Weismann K, Guerin DM, Parslew R. Acne keloidalis in caucasian patients on cyclosporin following organ transplantation. Br J Dermatol. 2000;143:465-467. 39. Grunwald MH, Ben-Dor D, Livni E, Halevy S. Acne keloidalis-like lesions on the scalp associated with antiepileptic drugs. Int J Dermatol. 1990;29:559-561. 40. Kelly AP. Pseudofolliculitis barbae and acne keloidalis nuchae. Dermatol Clin. 2003;21:645-653. 41. Sperling LC, Homoky C, Pratt L, Sau P. Acne keloidalis is a form of primary scarring alopecia. Arch Dermatol. 2000;136:479-484. 42. Califano J, Miller S, Frodel J. Treatment of occipital acne keloidalis by excision followed by secondary intention healing. Arch Facial Plast Surg. 1999;1:308311. 43. Gloster HM Jr. The surgical management of extensive cases of acne keloidalis nuchae. Arch Dermatol. 2000;136:1376-1379. 44. Perry PK, Cook-Bolden FE, Rahman Z, Jones E, Taylor SC. Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. J Am Acad Dermatol. 2002;46(2 Suppl):S113-119. 45. Khumalo NP, Callender VD. African hair. In: Blume-Peytavi U, Tosti A, Whiting DA, Trüeb R. eds. Hair growth and disorders. Leipzig: Springer. 2008:483-497. 46. Winter H, Schissel D, Parry DA, et al. An unusual Ala12Thr polymorphism in the 1A alpha-helical segment of the companion layer-specific keratin K6hf: evidence for a risk factor in the etiology of the common hair disorder pseudofolliculitis barbae. J Invest Dermatol. 2004;122:652-657. 47. Chuh A, Zawar V. Epiluminescence dermatoscopy enhanced patient compliance and achieved treatment success in pseudofolliculitis barbae. Australas J Dermatol. 2006;47:60-62. 48. Bridgeman-Shah S. The medical and surgical therapy of pseudofolliculitis barbae. Dermatol Ther. 2004;17:158-163. 49. Kligman AM, Mills OH Jr. Pseudofolliculitis of the beard and topically applied tretinoin. Arch Dermatol. 1973;107:551-552. 50. Cook-Bolden FE, Barba A, Halder R, Taylor S. Twice-daily applications of benzoyl peroxide 5%/clindamycin 1% gel versus vehicle in the treatment of pseudofolliculitis barbae. Cutis. 2004;73(6 Suppl):18-24. 51. Perricone NV. Treatment of pseudofolliculitis barbae with topical glycolic acid: a report of two studies. Cutis. 1993;52:232-235 52. Moraes MS, Godoy-Martínez P, Alchorne MM, Boatto HF, Fischman O. Incidence of tinea capitis in São Paulo, Brazil. Mycopathologia. 2006;162:9195. 53. Seebacher C, Abeck D, Brasch J, et al. Tinea capitis. J Dtsch Dermatol Ges. 2006;4:1085-1091. 54. Ilkit M. Favus of the scalp: an overview and update. Mycopathologia.

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LEGENDS Figure 1: Lichen planus pilaris: alopecic patch affecting the parietal region and vertex. Figure 2: Lichen planus pilaris: multiple alopecic patches at the vertex. Perifollicular erythema and scaling at the hair-bearing margin, indicating disease activity. Figure 3: Dermoscopy of lichen planus pilaris: perifollicular scaling with some scales forming tubular structures around the emerging hair shaft. Figure 4: Frontal fibrosing alopecia: frontotemporal band of alopecia – marked contrast between the smooth and shiny skin of the recession area and numerous facial papules corresponding to vellus follicle involvement Figure 5: Frontal fibrosing alopecia: detail of the facial papules. Figure 6: Dermoscopy of frontal fibrosing alopecia: mild perifollicular scaling. Figure 7: Discoid lupus erythematosus: atrophic patches on the scalp, ear and face Figure 8: Discoid lupus erythematosus: large atrophic alopecic patch at the vertex region with important dyschromia. Figure 9: Dermoscopy of discoid lupus erythematosus: dyschromia and prominent follicular red dots. Figure 10: Dermoscopy of discoid lupus erythematosus: alopecic patch with milky red areas and follicular plugging. The absence of pigmented network indicates destruction of the interfollicular architecture. Speckled blue-grey dots are also present. Figure 11: Folliculitis decalvans: Large lesion of at the vertex and occipital area. There is marked hair tufting with peripheral erythema. Figure 12: Trichoscopy of folliculitis decalvans: hair tufts with perifollicular hyperplasia Figure 13: Dissecting folliculitis: large erythematous nodules on the occipital area

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with few remaining hairs. Figure 14: Trichoscopy of dissecting folliculitis: erythema in the background with yellow dots, black dots and empty follicular openings Figure 15: Acne keloidalis nuchae: reddish brown dome-shaped papules on the nape of the neck Figure 16: Pseudofollicuitis barbae in a woman Figure 17: Dermoscopy of pseudofollicuitis barbae Figure 18: Tinea capitis: round patch of alopecia with intense scaling Figure 19: Tinea capitis: patch with vesico-erythematous borders Figure 20: Tinea capitis: inflammatoty lesions – kerion Figure 21: Trichoscopy of tinea capitis: comma hairs and corkscrew hairs in a lesion of tinea capitis. Figure 22: Tinea barbae: patch with erythematous borders Figure 23: Tinea faciei: single lesion of tinea faciei Figure 24: Tinea faciei: multiple and confluent patches of tinea faciei, with crusted and erythematous borders and hypopigmented and scaly centers.

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