OBSERVATION 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 Background: Granulomatous periorificial dermatitis is limiting, and were not associated with systemic involve- a well-recognized entity presenting most commonly in ment. Resolution seemed to be hastened with the use of prepubertal children as yellow-brown papules limited to systemic antibiotic therapy in 4 of the 5 patients. the perioral, perinasal, and periocular regions. The con- dition is self-limiting and is not associated with sys- Conclusions: Extrafacial lesions can occur in granulo- temic involvement. matous periorificial dermatitis and do not appear to ad- versely affect the duration, response to therapy, or risk Observations: We reviewed the medical charts of 5 of extracutaneous manifestations. Overly aggressive evalu- healthy children presenting with extrafacial granuloma- ation and inappropriate systemic therapy should be tous papules in addition to the typical periorificial avoided. papules. These extrafacial lesions were clinically and histologically identical to the facial lesions, were self- Arch Dermatol. 2002;138:1354-1358 1 N 1970, Gianotti et al described REPORT OF CASES 5 children with monomorphic perioral papules that showed a CASE 1 granulomatous pattern when le- sional biopsy sections were ex- A 23-month-old white boy with no history Iamined. Since then, several additional pa- of skin disease developed lesions on his right tients have been reported and the condition leg at the injection site 2 weeks after vari- has been variably called Gianotti-type peri- cella vaccination. During the subsequent oral dermatitis, sarcoidlike granulomatous month, lesions became widely dissemi- dermatitis, facial Afro-Caribbean child- nated, but were asymptomatic and had never hood eruption (FACE), granulomatous peri- been associated with fever, weight loss, oral dermatitis, and, most recently, granu- cough, shortness of breath, or ocular com- lomatous periorificial dermatitis (GPD).2-9 plaints. He was otherwise healthy and there All affected patients have been healthy pre- was no family history of similar skin le- pubertal children, and the eruption was sions. The skin lesions were initially treated confined to the skin surrounding the with 0.1% triamcinolone acetonide oint- mouth, nose, and eyes in 56 of 59 pa- ment for 3 weeks without improvement. From the Department of tients described.1-14 The 3 patients with The findings from the physical ex- Dermatology, University of extrafacial involvement who were de- amination 4 months after the onset of the Alabama at Birmingham scribed in the literature had lesions on the rash showed an active boy with thou- (Drs Urbatsch and Elewski); neck, upper trunk, extensor wrists, and sands of discrete 1- to 3-mm red to yellow- Department of Pediatric vaginal area.14,15 brown papules on his scalp, face, trunk, Dermatology, University of We describe 5 prepubertal children and extremities (Figure 3). The highest California at San Francisco with periorificial granulomatous derma- concentration of lesions was around his (Drs Frieden and Williams); Figure 4 and Department of Pediatric titis, but with extensive extrafacial in- mouth, nose, and eyes ( ). The Dermatology, Northwestern volvement as well. The clinical character- rest of the physical examination findings University Medical School, istics of our 5 patients and the 3 cases from were normal. Chicago, Ill (Drs Mancini the literature are summarized in Table 1. The examination of 3 serial lesional and Paller). Two of the cases are outlined herein. biopsy specimens showed noncaseating (REPRINTED) ARCH DERMATOL / VOL 138, OCT 2002 WWW.ARCHDERMATOL.COM 1354 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Table 1. Demographic and Clinical Data of Patients With Extrafacial Periorificial Granulomatous Dermatitis Patient No./ Extracutaneous Treatment Sex/Age, y Distribution Involvement Histologic Features Treatment Duration, mo* 1/M/2 Face, scalp, trunk, None Perifollicular granulomas Topical triamcinolone acetonide; 3wk;2mo extremities oral erythromycin estolate 2/F/12 Face, scalp, neck, ears Blepharitis Perifollicular granulomas Topical desonide; oral hydroxychloroquine 2wk;2mo;6wk sulfate, oral cyclosporine; oral minocycline, topical metronidazole 3/F/8 Face, upper trunk, Blepharitis, Perifollicular granulomatous Oral azithromycin, 1mo extremities lower tarsal infiltrate topical metronidazole papules 4/F/6 Face, labia majora None No data Oral and topical erythromycin 2 mo 5/F/8 Face, arms, abdomen, None Perifollicular granulomatous Oral erythromycin, Unknown labia majora† infiltrate topical metronidazole 6/M/415 Face, neck, upper trunk None Perifollicular granulomatous None 3 mo infiltrate 7/F/814 Face, ears, None Perifollicular granulomatous Oral macrolide antibiotic 6 mo extensor wrists infiltrate 8/F/814 Face, labia majora None Perifollicular granulomatous Oral macrolide antibiotic 6 mo infiltrate *Time to resolution after treatment was started. †See Figure 1 and Figure 2. Figure 1. Confluent erythematous scaly papules around the mouth and nose of patient 5. granulomas in the dermis, many alongside the hair fol- licles (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 ophthal- mologic examination were normal and a purified pro- tein derivative test was nonreactive. Because of the resemblance of the patient’s condi- tion to GPD, oral erythromycin estolate, 125 mg 3 times daily, was administered. This treatment resulted in dra- matic flattening of the lesions after 1 month of therapy and resolution of all lesions 6 months after the initia- tion of therapy and left mild atrophodermic scarring. The erythromycin regimen was tapered and discontinued af- Figure 2. Small dome-shaped papules and erythema on the labia majora of ter 9 months of treatment. Within 1 month after discon- patient 5. tinuation of the erythromycin, the lesions began to re- cur, once again initially at the site of the varicella vaccination. Treatment with oral erythromycin es- CASE 2 tolate, 125 mg 3 times daily, was restarted and again re- sulted in flattening of the lesions within 1 month. The A 12-year-old white girl with no significant medical his- resolved lesions left shallow pitted scars that resembled tory had a 1-year history of diffuse hair thinning. Two follicular atrophoderma (Figure 6). months later, she also developed hundreds of nonpru- (REPRINTED) ARCH DERMATOL / VOL 138, OCT 2002 WWW.ARCHDERMATOL.COM 1355 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/27/2021 Figure 3. Thousands of discrete reddish brown papules on the trunk and extremities of patient 1. The confluence of these lesions periorificially is striking. Figure 5. Noncaseating perifollicular granulomas on histologic examination of a papule from the trunk of patient 1 (hematoxylin-eosin, original magnification ϫ100). Figure 4. Diffuse reddish brown papules were most concentrated in the perioral region of patient 1. ritic 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 previ- ous year, but had never experienced any visual distur- bances. There was no family history of skin lesions, eye abnormalities, or hair loss. The results of a physical examination 14 months af- ter the onset of hair loss showed diffuse thinning of her scalp hair that was most pronounced on the vertex of the Figure 6. Resolution of the lesions on the trunk of patient 1 after the scalp, with widening of the central part. Scarring was not initiation of therapy with erythromycin estolate. The shallow pitted scarring apparent. She had hundreds of discrete flesh-colored pap- resembles follicular atrophoderma. ules with varying amounts of surrounding erythema and scale, on her scalp, face, ears, and neck. The facial le- showed no abnormalities. A chest x-ray film, results of sions were predominantly located periorally. The re- pulmonary function tests, and an electrocardiogram were sults of the examination also disclosed erythema and scale also normal. along the margins of her eyelids and shotty posterior cer- The patient was initially treated with desonide lo- vical lymphadenopathy. tion for 2 weeks, with worsening of the skin lesions. A Four lesional skin biopsy specimens were obtained presumptive diagnosis of sarcoidosis was made and oral from the scalp and neck; all showed granulomas in the hydroxychloroquine sulfate, 200 mg daily, and oral cy- dermis, mostly around the hair follicles (Figure 7). Fo- closporine, 150 mg daily, were administered. Medica- cal epidermal spongiosis overlay some of the hair fol- tions were discontinued after 2 months of therapy when licles. The results of special stains and cultures were nega- no improvement occurred. Because of the resemblance tive for mycobacteria and fungus. to granulomatous perioral dermatitis, oral minocycline Laboratory evaluation, including a complete blood hydrochloride, 100 mg twice daily, and topical 0.75% met- cell count, chemistry panel, calcium level, erythrocyte ronidazole, administered twice daily, were initiated, lead- sedimentation rate, VDRL test, thyroid function