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 systemic antibiotics (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 than the 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 steroidresponsive eruption around the mouth, nose, and/or eyes that worsens when the topical corticosteroid is discontinued. Dependency on the use of the topical corticosteroid may develop as

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, and polymorphonuclear 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

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 may suffice.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 2 3 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 5aminolevulinic acid has shown promise for treating perioral dermatitis in one report.36 PREVENTION

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