Chapter 82.Perioral Dermatitis

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    Chapter 82Perioral Dermatitis

    Leslie P. Lawley & Sareeta R.S. Parker

    PERIORAL DERMATITIS AT A GLANCE

    Inflammatory skin disorder of young women and children. Small papules, vesicles, and pustules in perioral, periorbital, and/or perinasal

    distribution.

    Treatment: stop topical corticosteroid use; initiate 2- to 3-month course of systemicantibiotics (tetracycline family or erythromycin) and/or topical metronidazole

    Perioral dermatitis is characterized by small, discrete papules and pustules in a periorificial

    distribution, predominantly around the mouth. Because this condition can involve areas other thanthe perioral region, the term periorificial dermatitis has been proposed for this disorder.1,2 The

    classic presentation is an eruption with overlapping features of an eczematous dermatitis and an

    acneiform eruption. Although initially described in young women of 1525 years of age, perioral

    dermatitis is now recognized to occur in children as well.3 A subset of perioral dermatitis shows

    granulomas when lesional skin is examined histologically. Several names have been used to describe

    this granulomatous form of perioral dermatitis, including granulomatous perioral dermatitis, facial

    Afro-Caribbean childhood eruption, and granulomatous periorificial dermatitis.

    HISTORICAL ASPECTS

    The first reports describing perioral dermatitis appeared in the 1950s; various names were given to

    the condition, however, there was a lack of defining clinical criteria. In 1957, Frumess and Lewis

    described a light sensitive seborrheid that is generally accepted as the first account of what was

    later termed perioral dermatitis by Mihan and Ayres in 1964.6,7 Later descriptions by Cochran and

    Thomson8 and Wilkinson, Kirton, and Wilkinson9 further defined this disorder, and more recently

    the term periorificial dermatitis has been proposed.2 The condition was first described in children in

    the late 1960s.

    EPIDEMIOLOGY

    Adult perioral dermatitis predominantly affects women. Pediatric perioral dermatitis may have a

    slight female preponderance and is seen equally among those of different races.1,10 The

    granulomatous form of perioral dermatitis has been reported mostly in children of prepubertal age.5

    Perioral dermatitis can occur as early as 6 months.1 An increased prevalence in African-American

    children has been reported, but more recent reviews do not support this finding.2,11

    ETIOLOGY AND PATHOGENESIS

    A relationship of perioral dermatitis to the misuse of topical corticosteroids (fluorinated or

    nonfluorinated) has been well established.12 Patients often reveal a history of an acute steroid-

    responsive eruption around the mouth, nose, and/or eyes that worsens when the topicalcorticosteroid is discontinued. Dependency on the use of the topical corticosteroid may develop as

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    the patient repeatedly treats the recurrent eruption. In other cases, the condition may worsen with

    the application of topical corticosteroids, especially in the granulomatous variant of perioral

    dermatitis, which usually occurs in prepubertal children.2 Perioral dermatitis has been reported in

    patients using inhaled corticosteroids13 and with inadvertent facial exposure to topical

    corticosteroids.14 However, perioral dermatitis is not always linked to topical corticosteroids.9 The

    exact cause of perioral dermatitis in these other cases is unclear. Although isolated reports of

    affected siblings exist,2,15 no clear genetic predisposition has been noted, nor have specific

    environmental exposures been consistently implicated. Of note, the disease is predominant in young

    women yet no link to hormonal causes has been found. The initial reports of photosensitivity by

    Frumess and Lewis6 were not further substantiated, nor were theories of microbiologic causes such

    as infection with Candida, fusiform bacteria, or Demodex folliculorum.16 Cases of allergic contact

    with fluorides or other components in toothpaste and dentifrices have also been reported, however,

    use of these agents after clearing of the perioral dermatitis without further eruption has also been

    described. Patch testing in a small series of patients led to few positive results, and these were not

    considered relevant.9

    In the past, authors have considered the relationship of perioral dermatitis to acne rosacea,

    however, the clinical features are distinct (see Section Differential Diagnosis). In perioral

    dermatitis, the histopathologic findings are variable and are dependent on the form of perioral

    dermatitis. In a histopathologic review of 26 patients with the nongranulomatous form, follicular

    spongiosis and eczematous changes were prominent features, suggesting that perioral dermatitis is

    distinct from rosacea.17 A lymphohistiocytic infiltrate and occasional plasma cells were noted in a

    perifollicular and perivascular distribution in this series. In granulomatous perioral dermatitis,

    histopathology demonstrates follicular hyperkeratosis, edema and vasodilatation in the papillary

    dermis, perivascular and parafollicular infiltrates of lymphocytes, histiocytes, andpolymorphonuclear leukocytes with occasional epithelioid granulomas and giant cells, similar to the

    histopathologic changes in acne rosacea.5,18

    CLINICAL FINDINGS

    The primary lesions of perioral dermatitis are discrete and grouped erythematous papules, vesicles,

    and pustules (Figs. 82-1 and 82-2). The lesions are often symmetric but may be unilateral and appear

    in the perioral, perinasal, and/or periocular regions (Figs. 82-2 and 82-3 and eFigs. 82-3.1 and 82-3.2

    in online edition). In a retrospective review of 79 children with perioral dermatitis, isolated perioral

    involvement was present in only 39%, and in rare cases nonperioral regions were involved

    exclusively.1 Background erythema and/or scale may be present. A distinct 5-mm clear zone at the

    vermilion edge is well described (Fig. 82-2). The granulomatous variant of perioral dermatitis

    presents with small flesh-colored, erythematous, or yellowbrown papules, some with confluence,

    and shares the distribution of perioral dermatitis in adults (Fig. 82-3). In addition, lesions have been

    reported to appear on the ears, neck, scalp, trunk, labia majora, and extremities

    Occasionally, an associated burning sensation or itching is reported, and intolerance to moisturizers

    and other topical products is described.1,9 In a few cases of granulomatous perioral dermatitis, an

    associated blepharitis or conjunctivitis has been reported.11 Systemic findings and regional

    lymphadenopathy are absent.

    DIFFERENTIAL DIAGNOSIS

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    The differential diagnosis of nongranulomatous and granulomatous perioral dermatitis is outlined in

    Box 82-1.1924 Both forms of perioral dermatitis lack systemic symptoms and a thorough history

    and physical examination are generally sufficient to establish the diagnosis. However, in some cases

    histopathological evaluation of lesional skin, chest radiography, and/or ophthalmologic examination

    may be necessary, particularly with the granulomatous variant.11 Sarcoidosis in young children is

    rare and often accompanied by systemic signs and symptoms such as weight loss, fatigue, joint

    pains, lymphadenopathy, and uveitis.5,25 At least some of the reported cases of sarcoidosis in young

    children represent Blau syndrome with underlying mutations in CARD15/NOD2 (see Chapter 134).

    COMPLICATIONS

    The majority of cases of perioral dermatitis and granulomatous perioral dermatitis resolve without

    sequelae or relapse. However, there are rare reports of scarring

    PROGNOSIS AND CLINICAL COURSE

    Perioral dermatitis is usually a self-limited disorder that evolves over a few weeks and resolves over

    months or rarely years. The condition may take on a waxing and waning course, often with a

    tendency to progress (granulomatous form). If treated with topical corticosteroids alone, recurrent

    episodes on withdrawal of therapy or with continuing therapy are typical. With appropriate

    intervention the condition resolves with rare recurrences.

    TREATMENT

    If topical corticosteroids are being used, they should be discontinued. If fluorinated corticosteroids

    are being applied, initial substitution with a low-potency hydrocortisone cream may minimize a flare

    of the dermatitis. Patients should be educated about the link between application of topical

    corticosteroids and exacerbation of the dermatitis.

    In most cases, effective therapy is oral tetracycline, doxycycline, or minocycline, for a course of 8 to

    10 weeks, with a taper over the last 2 to 4 weeks. In children under 8 years of age, nursing mothers,

    or tetracycline-allergic patients, oral erythromycin is recommended. Not uncommonly, patients

    require continued low-dose systemic antibiotic therapy for months or sometimes years to maintain

    control. In recalcitrant cases, isotretinoin may be considered.27

    Topical antibiotic therapy, most commonly with topical metronidazole, should be initiated

    concurrently with the systemic antibiotic. For milder cases, topical metronidazole alone maysuffice.1,28,29 In a retrospective review of 79 children, best outcomes were associated with the use

    of topical metronidazole, oral erythromycin, or both.1 Response is generally noted within 23

    months. Other options include topical clindamycin or erythromycin, topical sulfur-based

    preparations, and topical azelaic acid.30 Reports of successful use of topical calcineurin inhibitors

    exist, particularly in adults; however, caution is advised given the occasional reports of

    granulomatous eruptions after the use of these agents.3135 Ointment preparations should

    generally be avoided in the treatment of perioral dermatitis. Photodynamic therapy with topical 5-

    aminolevulinic acid has shown promise for treating perioral dermatitis in one report.36

    PREVENTION

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    The only widely accepted factor that may predispose to the development of perioral dermatitis is

    the use of topical corticosteroids. Avoiding facial skin exposure to these products may prevent the

    eruption in some case