BASICS
DESCRIPTION
- A common facial eruption of women and children
- Presents as tiny, flesh-colored, or erythematous monomorphic papules or pustules around the mouth with characteristic sparing of the area immediately adjacent to the vermilion border
- May also involve the periocular, perinasal, and glabellar regions of the face
- Etiology is unknown, but a relation to topical steroid use has been suggested.
- Without treatment, the course is usually fluctuating and chronic.
- Variants in children include granulomatous periorificial dermatitis (GPD) and facial Afro-Caribbean childhood eruption.
- Synonym(s): periorificial dermatitis (POD); chronic papulopustular facial dermatitis; granulomatous perioral dermatitis; light-sensitive seborrhea; lupus-like perioral dermatitis; papulopustular facial dermatitis; rosacea-like dermatitis; stewardess disease
EPIDEMIOLOGY
- Occurs worldwide, especially in fair-skinned populations
- Predominantly affects children between 6 months and 16 years and women aged 17 to 45 years
- ’ Ό90% of adult cases are in women (1).
- 55% of childhood cases occur in females (2).
- All races are affected, but the granulomatous form is more common in African American and dark-skinned children.
- Some believe the number of cases peaked in the 1960s to 1970s and decreased in the 1980s to 1990s when the side effects of topical steroids were recognized. Others believe that cases are still increasing.
Incidence
- Peak incidence is in the 2nd and 3rd decades of life.
- In children, peak incidence is in the prepubertal period.
Prevalence
- Represents ’ Ό2% of patients presenting to dermatology clinics (3)
- ’ Ό3% of children using inhalational steroids develop some form of POD (4).
ETIOLOGY AND PATHOPHYSIOLOGY
- Exposure to an irritant results in breakdown of the epidermal barrier and subsequent water loss and sensation of dryness; this encourages use of facial products and corticosteroids, which may worsen the condition.
- This inflammatory cycle eventually leads to clinical features of the disease.
- The exact cause is unknown, and there may be more than one contributing factor.
- The most widely cited factor implicated in POD is use of potent topical corticosteroids on the face.
- Inhaled corticosteroids, especially when used with a spacer and nebulizer or mask, have also been reported to cause POD.
- Intranasal steroids have been associated with POD beginning in the nasolabial folds.
- Two cases associated with systemic steroid use have been reported in children (5).
- Use of foundation, moisturizer, and night cream was associated with a 13-fold increased risk of POD in one study (6).
- Other factors are implicated but not proven:
- Drugs: oral contraceptives
- Toothpastes: fluoridated, tartar control, whitening
- Physical factors: UV light, heat, wind, salivary leakage
- Infectious factors: fusiform bacteria, Candida species, Demodex folliculorum
- Miscellaneous factors: atopy, GI disturbances, stress, contact allergy, lip-licking, immunosuppression, bubble gum, formaldehyde, varicella vaccination
Genetics
55% of pediatric patients with POD have a family history of atopy (6).
RISK FACTORS
See "Etiology and Pathophysiology. "
GENERAL PREVENTION
- Avoid using potent topical corticosteroids on the face.
- Avoid using excessive foundation, moisturizer, and night cream.
- Avoidance of tartar-control and whitening toothpastes may also be helpful.
COMMONLY ASSOCIATED CONDITIONS
Atopic dermatitis
DIAGNOSIS
HISTORY
- Patient reports a history of facial rash, which may improve with use of topical corticosteroids and flare with discontinuation.
- Affected skin may be pruritic or have a burning sensation.
- Course may be chronic and fluctuating.
- Recent topical, inhaled, or systemic steroid use in 72% of patients (7)
- May worsen with sun exposure
PHYSICAL EXAM
- Monomorphic, minute, flesh-colored to erythematous papules or pustules, sometimes surmounted with scale, in a perioral distribution, often asymmetrical, with sparing of the vermilion border 3 to 5 mm around the lips
- Granulomatous form can usually be distinguished clinically by the absence of pustules and the presence of yellow-brown papules.
- Common locations are alar creases, nasolabial folds, chin, periocular skin, eyelids, and glabella.
- Diameter of lesions is usually 1 to 2 mm.
- Distribution (3)
- Perioral area (39%)
- Perinasal (13%)
- Periocular (1%)
- Perioral and perinasal (14%)
- Perioral and periocular (6%)
- Perinasal and periocular (6%)
- Perioral, perinasal, and periocular (10%)
- Lupus-like variant diagnosed by yellowish discoloration of lesions on diascopy
DIFFERENTIAL DIAGNOSIS
- Acne vulgaris
- Contact dermatitis
- Rosacea
- Seborrheic dermatitis
- Lip-licker 's dermatitis
- Papular sarcoidosis
- Demodex infestation
- Acrodermatitis enteropathica
- Biotin deficiency
- Glucagonoma syndrome
- Xanthomas
- Eruptive syringomas
- Polymorphous light eruption
- Acne agminata
- Lupus miliaris disseminatus faciei
- Haber syndrome (familial rosacea-like dermatosis)
DIAGNOSTIC TESTS & INTERPRETATION
Diagnosis is usually clinical.
- No laboratory abnormalities are expected.
- Prick tests and IgE testing may show evidence of atopy but are not routinely done.
- Patch testing may be used to rule out contact dermatitis.
- Scrapings for Demodex mites
Diagnostic Procedures/Other
Skin biopsy is not routinely performed but can be helpful in atypical cases.
Test Interpretation
Histopathology of skin biopsy
- Mild, nonspecific inflammation with variable perifollicular or perivascular lymphohistiocytic infiltrate
- Eczematous changes, acanthosis, parakeratosis, and spongiosis
- Occasionally, follicular abscesses are seen.
- Older lesions may contain diffuse hypertrophy of connective tissue and hyperplasia of sebaceous follicles with occasional noncaseating granulomas in the dermis (1).
- Caseating granulomas are seen with granulomatous variant.
TREATMENT
GENERAL MEASURES
"Zero therapy " can be an effective treatment in adherent patients (3)[B]:
- Reduce use of facial soaps, makeup, and moisturizers, and wash with only water.
- Discontinue use of potent topical corticosteroids on the face.
- This may result in a flare.
- Flares may be decreased with use of less-potent topical steroids, calcineurin inhibitors, or IM steroid injection.
- Discontinue use of fluoridated, whitening, and tartar-control toothpaste.
MEDICATION
Based on disease severity and patient 's tolerance for adverse effects
First Line
- Severe disease
- Oral antibiotics
- Tetracycline has been proven as an effective treatment (3)[A].
- 250 to 500 mg BID for 8 to 10 weeks
- Dose may be halved after 3 to 4 weeks if improvement is noted.
- Contraindicated in children <8 years of age and pregnant women.
- Common side effects: photosensitivity, GI upset, rash, candidiasis, headache, dizziness, and tinnitus
- Doxycycline or minocycline (6)[C]
- 50 to 100 mg BID for 6 to 8 weeks
- Dose may be halved after 3 to 4 weeks if improvement is noted.
- Contraindicated in children <8 years of age and pregnant women.
- Common side effects of doxycycline: GI upset, esophagitis, joint pain, upper respiratory infection (URI) symptoms, rash, dysmenorrhea, candidiasis, headache, dizziness, photosensitivity, and elevated BUN
- Common side effects of minocycline: GI upset, lightheadedness, vertigo, ataxia, headache, fatigue, tinnitus, rash, urticaria, candidiasis, and hyperpigmentation
- Alternatively, doxycycline 40 mg/day modified-release capsule may achieve the anti-inflammatory effect without the microbial activity that promotes emergence of resistant bacterial strains (8)[C].
- Erythromycin (6)[C] may be used when tetracycline are contraindicated or not tolerated.
- 250 mg BID " TID for 6 to 8 weeks
- Dose may be halved after 3 to 4 weeks if improvement noted.
- May be used in children or pregnant women
- Common side effects: GI upset, diarrhea, rash, and urticaria
- Rarer serious side effects: erythema multiforme, pancreatitis, convulsions, QT prolongation, and ventricular arrhythmias
- Moderate disease
- Topical antibiotics
- May be slower than systemic antibiotics in clearing lesions
- Metronidazole cream 0.75% BID (3)[B]
- Erythromycin 2% gel, solution, or ointment BID (3)[B]
- Clindamycin 1% gel, lotion, or cream BID
- Mild disease
- Zero therapy (see "General Measures " ) (3)[B]
Second Line
- Severe disease
- Azithromycin (2) or clarithromycin (250 mg/day) may be used when tetracycline and erythromycin (3)[C] are contraindicated or not tolerated.
- Common side effects: GI upset, headache, rash, abnormal taste sensation, and urticaria
- Oral isotretinoin may be useful in granulomatous cases that are not responsive to a full dose of tetracycline (1)[C].
- 0.2 mg/kg initially, followed by 0.1 mg/kg after notable improvement
- Isotretinoin is a pregnancy Category X drug and may cause severe birth defects. Use with caution with in women of reproductive age. Physician and patient must register with iPLEDGE program.
- Common side effects: dry skin, cheilitis, arthralgias/myalgias, hypertriglyceridemia, elevated liver function tests (LFTs), hair loss, visual disturbances, depression, and tinnitus
- Pimecrolimus 1% cream may accelerate healing (3)[A], and tacrolimus 0.1% ointment use has been reported with success (3).
- Rare cases of lymphoma and skin malignancies have been reported. Avoid long-term use
- Moderate disease
- Topical therapies
- Other antiacne drugs, such as sodium sulfacetamide (2), azelaic acid, and adapalene (3), may be helpful.
- Ichthyol (ammonium bituminosulfonate) (3)
ADDITIONAL THERAPIES
A number of other therapies have been reported in the literature, including photodynamic therapy with 5-aminolevulinic acid (3), radiotherapy, and liquid nitrogen. There has also been a case reported of oral metronidazole (250 mg BID) resulting in complete clearance of GPD without scarring after 1 month of use (9). Most recently, a randomized controlled trial showed significant improvement after 4 weeks of treatment with praziquantel 3% ointment BID (10).
COMPLEMENTARY & ALTERNATIVE MEDICINE
Compress with chamomile tea or physiologic solution may be used if the patient struggles with zero therapy (1).
ONGOING CARE
DIET
No restrictions
PATIENT EDUCATION
- Lesions may take many weeks to resolve.
- Symptoms may temporarily worsen, especially with discontinuance of steroids.
- Recurrence is rare.
- Topical corticosteroid use on the face should be avoided in the future.
PROGNOSIS
- Most cases resolve without recurrence.
- Oral or topical antibiotics usually lead to remission within 6 to 10 weeks.
- Untreated disease may persist for months to years and be characterized by unpredictable flares.
- Disease is not associated with significant morbidity.
COMPLICATIONS
- Most cases resolve without complications.
- Emotional stress can result from bothersome appearance of lesions and chronic course of disease.
- Scarring may be problematic with lupus-like variant.
REFERENCES
11 Lipozencic J, Ljubojevic S. Perioral dermatitis. Clin Dermatol. 2011;29(2):157 " 161.22 Goel NS, Burkhart CN, Morrell DS. Pediatric periorificial dermatitis: clinical course and treatment outcomes in 222 patients. Pediatr Dermatol. 2015;32(3):333 " 336.33 Wollenberg A, Bieber T, Dirschka T, et al. Perioral dermatitis [in English, German]. J Dtsch Dermatol Ges. 2011;9(5):422 " 427.44 Henningsen E, Bygum A. Budesonide-induced periorificial dermatitis presenting as chalazion and blepharitis. Pediatr Dermatol. 2011;28(5):596 " 597.55 Clementson B, Smidt AC. Periorificial dermatitis due to systemic corticosteroids in children: report of two cases. Pediatr Dermatol. 2012;29(3):331 " 332.66 Vanderweil SG, Levin NA. Perioral dermatitis: it 's not every rash that occurs around the mouth. Dermatol Nurs. 2009;21(6):317 " 320, 353.77 Nguyen V, Eichenfield LF. Periorificial dermatitis in children and adolescents. J Am Acad Dermatol. 2006;55(5):781 " 785.88 Rosso JQ. Management of papulopustular rosacea and perioral dermatitis with emphasis on iatrogenic causation or exacerbation of inflammatory facial dermatoses: use of doxycycline-modified release 40mg capsule once daily in combination with properly selected skin care as an effective therapeutic approach. J Clin Aesthet Dermatol. 2011;4(8):20 " 30.99 Rodriguez-Caruncho C, Bielsa I, Fernandez-Figueras MT, et al. Childhood granulomatous periorificial dermatitis with a good response to oral metronidazole. Pediatr Dermatol. 2013;30(5):e98 " e99.1010 Bribeche MR, Fedotov VP, Jillella A, et al. Topical praziquantel as a new treatment for perioral dermatitis: results of a randomized vehicle-controlled pilot study. Clin Exp Dermatol. 2014;39(4):448 " 453.
ADDITIONAL READING
- Hafeez ZH. Perioral dermatitis: an update. Int J Dermatol. 2003;42(7):514 " 517.
- Kim YJ, Shin JW, Lee JS, et al. Childhood granulomatous periorificial dermatitis. Ann Dermatol. 2011;23(3):386 " 388.
- Peralta L, Morais P. Perioral dermatitis " the role of nasal steroids. Cutan Ocul Toxicol. 2012;31(2):160 " 163.
- Weber K, Thurmayr R. Critical appraisal of reports on the treatment of perioral dermatitis. Dermatology. 2005;210(4):300 " 307.
CODES
ICD10
L71.0 Perioral dermatitis
ICD9
695.3 Rosacea
SNOMED
- 238751002 Perioral dermatitis (disorder)
- 403655004 Perioral dermatitis due to corticosteroid (disorder)
- 238583005 Saliva-induced contact dermatitis (disorder)
CLINICAL PEARLS
- Suspect POD when an acneiform eruption involves the perioral, perinasal, or periocular areas with characteristic sparing of the skin immediately adjacent to the vermilion border.
- Ask patients about a history of recent corticosteroid use, facial product use, and use of fluoridated, whitening, or tartar-control toothpastes.
- Substitution of low-potency steroids may allow patients to discontinue higher potency steroids while avoiding a rebound flare.