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Extrafacial and Generalized Granulomatous Periorificial Dermatitis
Amy J. Urbatsch, MD;
Ilona Frieden, MD;
Mary L. Williams, MD;
Boni E. Elewski, MD;
Anthony J. Mancini, MD;
Amy S. Paller, MD
Arch Dermatol. 2002;138:1354-1358.
ABSTRACT
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Background Granulomatous periorificial dermatitis is a well-recognized entity presenting
most commonly in prepubertal children as yellow-brown papules limited to the
perioral, perinasal, and periocular regions. The condition is self-limiting
and is not associated with systemic involvement.
Observations We reviewed the medical charts of 5 healthy children presenting with
extrafacial granulomatous papules in addition to the typical periorificial
papules. These extrafacial lesions were clinically and histologically identical
to the facial lesions, were self-limiting, and were not associated with systemic
involvement. Resolution seemed to be hastened with the use of systemic antibiotic
therapy in 4 of the 5 patients.
Conclusions Extrafacial lesions can occur in granulomatous periorificial dermatitis
and do not appear to adversely affect the duration, response to therapy, or
risk of extracutaneous manifestations. Overly aggressive evaluation and inappropriate
systemic therapy should be avoided.
INTRODUCTION
IN 1970, Gianotti et al1 described 5
children with monomorphic perioral papules that showed a granulomatous pattern
when lesional biopsy sections were examined. Since then, several additional
patients have been reported and the condition has been variably called Gianotti-type perioral dermatitis, sarcoidlike granulomatous dermatitis,
facial Afro-Caribbean childhood eruption (FACE), granulomatous perioral dermatitis, and, most recently, granulomatous periorificial
dermatitis (GPD).2-9 All
affected patients have been healthy prepubertal children, and the eruption
was confined to the skin surrounding the mouth, nose, and eyes in 56 of 59
patients described.1-14 The
3 patients with extrafacial involvement who were described in the literature
had lesions on the neck, upper trunk, extensor wrists, and vaginal area.14-15
We describe 5 prepubertal children with periorificial granulomatous
dermatitis, but with extensive extrafacial involvement as well. The clinical
characteristics of our 5 patients and the 3 cases from the literature are
summarized in Table 1
(See Figure 1 and
Figure 2). Two of the cases are outlined herein.
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Table 1. Demographic and Clinical Data of Patients With Extrafacial
Periorificial Granulomatous Dermatitis
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Figure 1. Confluent erythematous scaly papules
around the mouth and nose of patient 5.
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Figure 2. Small dome-shaped papules and
erythema on the labia majora of patient 5.
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REPORT OF CASES
CASE 1
A 23-month-old white boy with no history of skin disease developed lesions
on his right leg at the injection site 2 weeks after varicella vaccination.
During the subsequent month, lesions became widely disseminated, but were
asymptomatic and had never been associated with fever, weight loss, cough,
shortness of breath, or ocular complaints. He was otherwise healthy and there
was no family history of similar skin lesions. The skin lesions were initially
treated with 0.1% triamcinolone acetonide ointment for 3 weeks without improvement.
The findings from the physical examination 4 months after the onset
of the rash showed an active boy with thousands of discrete 1- to 3-mm red
to yellow-brown papules on his scalp, face, trunk, and extremities (Figure 3). The highest concentration of lesions
was around his mouth, nose, and eyes (Figure
4). The rest of the physical examination findings were normal.
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Figure 3. Thousands of discrete reddish
brown papules on the trunk and extremities of patient 1. The confluence of
these lesions periorificially is striking.
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Figure 4. Diffuse reddish brown papules
were most concentrated in the perioral region of patient 1.
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The examination of 3 serial lesional biopsy specimens showed noncaseating
granulomas in the dermis, many alongside the hair follicles (Figure 5). The results of special stains for acid-fast bacilli and
fungus were negative, and cultures yielded no organisms. A polarization test
for foreign material was negative. A chest x-ray film and findings of an ophthalmologic
examination were normal and a purified protein derivative test was nonreactive.
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Figure 5. Noncaseating perifollicular granulomas
on histologic examination of a papule from the trunk of patient 1 (hematoxylin-eosin,
original magnification x100).
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Because of the resemblance of the patient's condition to GPD, oral erythromycin
estolate, 125 mg 3 times daily, was administered. This treatment resulted
in dramatic flattening of the lesions after 1 month of therapy and resolution
of all lesions 6 months after the initiation of therapy and left mild atrophodermic
scarring. The erythromycin regimen was tapered and discontinued after 9 months
of treatment. Within 1 month after discontinuation of the erythromycin, the
lesions began to recur, once again initially at the site of the varicella
vaccination. Treatment with oral erythromycin estolate, 125 mg 3 times daily,
was restarted and again resulted in flattening of the lesions within 1 month.
The resolved lesions left shallow pitted scars that resembled follicular atrophoderma
(Figure 6).
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Figure 6. Resolution of the lesions on the
trunk of patient 1 after the initiation of therapy with erythromycin estolate.
The shallow pitted scarring resembles follicular atrophoderma.
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CASE 2
A 12-year-old white girl with no significant medical history had a 1-year
history of diffuse hair thinning. Two months later, she also developed hundreds
of nonpruritic lesions on her face and neck. She denied fever, weight loss,
cough, shortness of breath, or arthralgias. She had had recurrent episodes
of blepharitis during the previous year, but had never experienced any visual
disturbances. There was no family history of skin lesions, eye abnormalities,
or hair loss.
The results of a physical examination 14 months after the onset of hair
loss showed diffuse thinning of her scalp hair that was most pronounced on
the vertex of the scalp, with widening of the central part. Scarring was not
apparent. She had hundreds of discrete flesh-colored papules with varying
amounts of surrounding erythema and scale, on her scalp, face, ears, and neck.
The facial lesions were predominantly located periorally. The results of the
examination also disclosed erythema and scale along the margins of her eyelids
and shotty posterior cervical lymphadenopathy.
Four lesional skin biopsy specimens were obtained from the scalp and
neck; all showed granulomas in the dermis, mostly around the hair follicles
(Figure 7). Focal epidermal spongiosis
overlay some of the hair follicles. The results of special stains and cultures
were negative for mycobacteria and fungus.
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Figure 7. Small, well-formed dermal perifollicular
granulomas on histologic examination of a papule from the scalp of patient
2 (hematoxylin-eosin, original magnification x200).
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Laboratory evaluation, including a complete blood cell count, chemistry
panel, calcium level, erythrocyte sedimentation rate, VDRL test, thyroid function
tests, and serum levels of antinuclear antibody and testosterone, showed no
abnormalities. A chest x-ray film, results of pulmonary function tests, and
an electrocardiogram were also normal.
The patient was initially treated with desonide lotion for 2 weeks,
with worsening of the skin lesions. A presumptive diagnosis of sarcoidosis
was made and oral hydroxychloroquine sulfate, 200 mg daily, and oral cyclosporine,
150 mg daily, were administered. Medications were discontinued after 2 months
of therapy when no improvement occurred. Because of the resemblance to granulomatous
perioral dermatitis, oral minocycline hydrochloride, 100 mg twice daily, and
topical 0.75% metronidazole, administered twice daily, were initiated, leading
to resolution of all skin lesions within 6 weeks without scarring. The minocycline
was tapered during a 4-month period. The patient continues to use the 0.75%
metronidazole lotion twice a day. Her scalp hair is still thin, but no further
hair loss or new skin lesions have been noted 6 months after the discontinuation
of the minocycline.
COMMENT
Granulomatous periorificial dermatitis is thought to be a less common
variant of perioral dermatitis. Perioral dermatitis is an eruption characterized
by grouped red papules, pustules, or papulovesicles and diffuse erythema and
scaling around the mouth, nose, and eyes that can be seen in children.16 The lack of pustules and the presence of discrete
yellow-brown papules, less prominent erythema and scaling, and a perifollicular
granulomatous infiltrate on examination of a biopsy specimen can differentiate
GPD from perioral dermatitis.9 Most reported
cases of GPD have occurred in prepubertal children. The disease affects both
sexes almost equally. According to the literature, GPD is seen more commonly
in dark-skinned patients, but this observation may reflect population bias
and, in fact, all but 1 of our cases occurred in white patients.
The primary lesion is a discrete 1- to 3-mm dome-shaped papule that
is red or yellow-brown. In some instances erythema surrounds the papule and
overlying scale is present. As in classic periorificial dermatitis, the face
is always involved, with lesions concentrated around the mouth, nose, and
eyes. Some cases of GPD have described prominent involvement of the helices
of the ears as an important diagnostic feature.2, 6
Scarring is variable. The initial cases described by Gianotti et al1 and several subsequent authors2, 13-14 reported
the occurrence of small pitted scars after resolution of the papules. This
same type of scarring was seen in one of our patients (patient 1) and is likely
a result of the inflammatory process.
Although the histologic appearance alone is not diagnostic, cases in
which skin biopsy was performed have shown a dermal granulomatous infiltrate,
usually concentrated around the upper half of normal, nondisrupted hair follicles.
In some biopsy specimens, the infiltrate has been more diffuse, with multiple
epithelioid macrophages, lymphocytes, and giant cells; in others, well-formed
noncaseating granulomas are surrounded by lymphocytes. Focal epidermal spongiosis
is occasionally described. The results of special stains and cultures for
acid-fast bacilli and fungi are always negative.
The etiology of this condition is unknown. In some cases the eruption
was linked to an external allergic or irritant contactant. The essential oils
in bubble gum, antiseptic solution, formaldehyde, and black synthetic mesh
are among the incriminated agents.2-4,12 Topical
fluorinated corticosteroids have been incriminated in triggering and exacerbating
GPD.5 In patient 1, the eruption was temporally
and positionally related to varicella vaccination. Granulomatous periorificial
dermatitis may represent a nonspecific granulomatous response to a variety
of topical or systemic agents.
Granulomatous periorificial dermatitis is a benign and self-limited
condition without any systemic manifestations. Occasionally, blepharitis or
conjunctivitis is an associated finding, and blepharitis occurred in patients
2 and 3. Results of routine laboratory studies and ophthalmologic examination
are usually normal; a chest radiograph is likewise usually normal. Spontaneous
resolution usually occurs by a few months to 3 years after onset.1-15 The
administration of oral macrolides or tetracyclines, alone or in combination
with topical erythromycin, metronidazole, or sulfur-based lotions, hastens
resolution in most patients.
The differential diagnosis of small papules with granulomatous histologic
features in children includes sarcoidosis, fungal or mycobacterial infection,
familial juvenile systemic granulomatosis (Blau syndrome), and granulomatous
rosacea (Table 2). In typical
cases of GPD, these entities can be differentiated clinically. In cases with
extrafacial involvement, other disorders can be differentiated by a thorough
history and physical examination, a review of symptoms, examination of a chest
radiograph, an ophthalmologic examination, and the use of special stains and
cultures of tissue specimens for fungal or mycobacterial organisms.
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Table 2. Differential Diagnosis of Granulomatous Papules in Children
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The diagnosis of granulomatous rosacea deserves special consideration.
Both GPD and granulomatous rosacea present with red or yellow-brown, dome-shaped
facial papules and a perifollicular lymphohistiocytic or granulomatous infiltrate.
Other similarities include the occurrence of blepharitis or conjunctivitis,
extrafacial lesions, and a response to the tetracycline class of antibiotics
and topical metronidazole. Lesions have been described as involving the ears,
neck, axillae, trunk, and groin in granulomatous rosacea.17 The
major distinguishing features of granulomatous rosacea are the lack of a concentration
of periorificial lesions and the association of erythema, telangiectasias,
pustules, and edema, which are features of more typical rosacea.17 Rosacea
and granulomatous rosacea have been reported in children, but are uncommon.17 A case of GPD was reported in a girl who later developed
a more acneiform and telangiectatic form of rosacea (Judith Schiffner, MD,
oral communication, February 2001).8 Arguably,
GPD may actually be a variant of granulomatous rosacea and, in a subset of
patients, the first manifestation of a rosacea diathesis.
The 8 cases summarized in Table 1 extend the clinical spectrum of GPD; all of these children had
the classic facial periorificial papules, but also developed extrafacial lesions
that were clinically and histologically indistinguishable from the facial
lesions. Extensive involvement did not appear to change the duration of the
eruption, the response to treatment, or the risk of extracutaneous manifestations.
The diagnosis of GPD is important to consider in the child who presents with
grouped papules around the mouth, nose, and eyes as is typical of GPD, but
also in those with similar lesions in nonfacial areas. Overly aggressive evaluation
and inappropriate systemic therapy should be avoided. Just as perioral was expanded to periorificial because
lesions could occur around the nose and eyes, it may be necessary to further
expand this entity to include extrafacial lesions as well.
AUTHOR INFORMATION
Accepted for publication February 21, 2002.
Corresponding author: Amy J. Urbatsch, MD, Department of Dermatology,
University of Alabama at Birmingham, 700 18th St S, Suite 414, Birmingham,
AL 35233 (e-mail: amyu{at}uab.edu).
From the Department of Dermatology, University of Alabama at Birmingham
(Drs Urbatsch and Elewski); Department of Pediatric Dermatology, University
of California at San Francisco (Drs Frieden and Williams); and Department
of Pediatric Dermatology, Northwestern University Medical School, Chicago,
Ill (Drs Mancini and Paller).
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