Dermatol Sinica, March 2006 38

Granulomatous Periorificial Dermatitis in a Young WomanYu-Wen Li1 Ming-Tuo Chuan1, 2 Shu-Ling Hu1

Granulomatous periorificial dermatitis is a skin disease presenting predominantly in prepubertalblack-skinned children. It is characterized by tiny, monomorphous, papular eruptions around the mouth,nose and eyes. Extra-facial lesions in children had also been reported. These lesions are self-limited andresolved without scarring in most cases. We report a 28-year-old woman presented with pink to normalskin colored, discrete and coalescing papules ranging from 1 to 3 mm in diameter over the face, napeand bilateral forearms of 8 months duration. A biopsy on the right nasolabial fold showed dermal gran-uloma formation around hair follicles, composed of lymphocytes, epithelioid histiocytes and occasionalmultinucleated giant cells. Oral doxycycline and topical metronidazole gel were given and the skinlesions cleared after 2 months treatment.(Dermatol Sinica 24: 38-41, 2006)

Key words: Granulomatous periorificial dermatitis, Adult

28

8 1 3

( 24: 38-41,

2006)

From the Department of Dermatology, Cathay General Hospital;1 Excelsior Wellness Center2

Accept for publication: September 09, 2005Reprint requests: Ming-Tuo Chuan, M.D., Department of Dermatology, Cathay General Hospital, 280 Jen-Ai Rd. Sec.4, Taipei,Taiwan.TEL: 886-2-27082121 ext. 5078 FAX: 886-2-27074949 E-mail: tuo@seed.net.tw

39 Dermatol Sinica, March 2006

INTRODUCTIONIn 1970, Gianotti et al. first described five

children, ranging from 2 to 7 years old, who hadasymptomatic, distinctive normal skin colored"micronodular" eruption surrounding themouth.1 Biopsy specimens demonstrated a lym-phohistiocytic infiltration with occasional giantcells. In the literature, the condition has beenvariably called Gianotti-type perioral dermatitis,sarcoidlike granulomatous dermatitis, facialAfro-Caribbean childhood eruption (FACE),and childhood granulomatous perioral dermatitis.

CASE REPORTA 28-year-old healthy lady complained of

asymptomatic, papular skin eruption on theface, nape and bilateral forearms at our outpatient clinic in June, 2004. She claimed thatthe asymptomatic papular skin rash on the facehappened 4 years ago and disappeared sponta-

neously about a year later. Similar skin lesionwas noted again on her face about 8 months agowith progression to the nape and bilateral fore-arms gradually. Topical clindamycin, topicalsteroid, and oral Chinese herbal drugs were giv-en but in vain. She denied any topical or oralmedications prior to the onset of the skin rashes.Physical examination revealed multiple skinpapules over the paranasal, perioral, periocularareas, glabella, nape and bilateral forearms (Fig.1). There were many 1- to 3-mm, pink to nor-mal skin colored papules scattered discretely orgrouped and most concentrated on the paranasalarea with extension to the nostrils and involve-ment of the vermilion borders. Neither comedonor telangiectasia was noted. Laboratory data,chest X ray and KOH examination were all nor-mal without specific findings. A skin biopsyspecimen from the right nasolabial fold showedmild spongiosis, basal vacuolization and lym-

Fig. 1 (A) Multiple skin papuleson the paranasal, perioral,periocular areas, andglabella. (B) Closer view showedthat the skin lesions weremost concentrated on theparanasal areas withextension to the nostrilsand involvement of thevermilion

Fig. 2(A) Dermal granulomaformation around the hairfollicles. (H&E stain, x40) (B) A granuloma com-posed of lymphocytes,epithelioid histiocytesand occasional multinu-cleated giant cells. (H&Estain, x 200)

(A) (B)

(A) (B)

Dermatol Sinica, March 2006 40

phocyte exocytosis of inter- and intra-follicularepidermis. Dermal granuloma formation aroundthe hair follicles was noted and composed oflymphocytes, epithelioid histiocytes and occa-sional multinucleated giant cells. Mild dermalfibroplasia, interstitial lymphohistiocytic infil-tration and vascular dilatation were noted (Fig.2). Acid-fast, periodic acid-Schiff, and Giemsastains revealed no evidence of specific microor-ganisms. Granulomatous periorificial dermatitiswas diagnosed. Stop using all of her medica-tions was suggested. Oral doxycycline, 100 mgtwice a day combined with topical 0.75%metronidazole gel was given for 3 weeks.Improvement was noted. Metronidazole gel wascontinued for further 3 weeks and all the skinlesions disappeared without scarring and pig-mentation 2 months later. No recurrence wasnoted after 6 months follow-up.

DISCUSSIONGranulomatous periorificial dermatitis

(GPD) is an uncommon skin disease ocurredpredominantly in prepubertal children. Blackchildren were affected much more commonlythan children of other racial backgrounds.2 Bothgenders are equally affected.3 Arguably, it hasbeen proposed that GPD is a localized variant ofrosacea.4 However, some authors suggested that"granulomatous rosacea" does not associatewith persistent facial erythema, flushing, and isa misnomer in rosacea group.5 They suggest anew name "granulomatous facial dermatitis" tocategorize diseases including GPD, facial Afro-Caribbean childhood eruption (FACE), andlupus miliaris disseminatus faciei.5

In the literature, GPD is often consideredas a granulomatous variant of perioral dermati-tis.6 The classic perioral dermatitis consists ofpapular, pustular or papulovesicular lesions onan erythematous background. The skin lesionsare usually confined to the chin and nasolabialfolds and spare around the vermilion border. Inperioral dermatitis, most of the histopathologyreports have shown mild, nonspecific, subacuteinflammation with variable perifollicular orperivascular lymphohistiocytic infiltration and

occasional papillary edema.6 However, somecases with granulomatous changes have beenreported and were designated as GPD.1 GPD ischaracterized by a monomorphous, papulareruption occurring around the mouth, nose andeyes. The primary lesion is a discrete 1-3 mmdome-shaped flesh-colored, yellow-brown, orred papule. Slight scaling of the lesions or sur-rounding erythema may occur. Extrafaciallesions had been reported involving the trunk,extremities, labia majora and could be general-ized.7 Extrafacial involvement do not appear toadversely affect the duration, response to thera-py, or risk of extracutaneous manifestation.7

Whether classic type GPD or GPD with extrafa-cial involvement, in most cases the lesionshealed without sequelae and only occasionallythe disease resolved with pinpoint atrophic scarsor altered pigmentation.2, 8 Results of routinelaboratory studies and chest radiograghs areusually normal.3 Most of the GPD occurred inchildren. In the literature, GPD had beendescribed in a young woman with a persistenteruption around the mouth and chin. She wasunresponsive to conventional therapies, and oralisotretinoin was given. After 20 weeks treat-ment, the lesions cleared and left pitted, atroph-ic scars.4 Histological findings of GPD revealupper dermal and perifollicular granuloma for-mation. The granulomas consist of epithelioidhistiocytes, lymphocytes and occasional multin-ucleated giant cells. Focal epidermal spongiosisis also described.9, 10 In addition, special stainsshould be done to rule out possible infectiouscauses such as lupus vulgaris, atypicalmycobacterial infection, and late secondary ortertiary syphilis. The presence of lymphocyticinflammation can help distinguish GPD fromthe "naked " granulomas in cutaneous sarcoido-sis that typically lack inflammatory cells.2, 9 Theetiology of GPD is unknown. Topical corticos-teroid may induce or exacerbate both GPD andperioral dermatitis.9, 11 Some suggested it to bean unusual granulomatous inflammatoryresponse to allergens. The initial allergen maycause inflammation and a focal disruption of thefollicular wall, inciting a granulomatous reac-

41 Dermatol Sinica, March 2006

tion.9 Suspect inciting subject including essen-tial oils in bubble gum, formaldehyde, cosmeticpreparations, and antiseptic solutions.6,12 Theclinical differential diagnosis includes trichoep-ithelioma, cutaneous sarcoidosis, fungal ormycobacterial infection. However, these disor-ders could be differentiated by the detailed his-tory, review of systems, thorough physicalexamination, laboratory studies, and specialstains or tissue cultures of biopsy specimens.

Although the new name "granulomatousfacial dermatitis" was suggested to categorizediseases including GPD and lupus miliaris dis-seminatus faciei (LMDF) ; 5 clinically, LMDFpresents as multiple red to yellow-brownpapules, nodules affecting central area of theface with a remarkable preference for the eye-lids.13 Histopathological examination showstuberculoid granulomas usually with centralcaseation necrosis. As the name LMDF sug-gests, it was originally presumed to be anexpression of cutaneous tuberculosis or a tuber-culid due to its histopathological similarity.However, all other facts including absence ofbacilli in the lesions, not detectable ofMycobacterium tuberculosis by polymerasechain reaction were all against with the hypoth-esis of tuberculosis.13, 14 The lesions of LMDFare often disappeared spontaneously with ice-picking scars.14 The prognosis of GPD is goodand spontaneous resolution usually occurs by afew months to 3 years after onset.7 Although itis asymptomatic, patients seek for medical helpdue to disfiguring cosmetically. At first, topicalcorticosteroid should be strictly avoided. Themainstay of treatment includes administrationof oral macrolides or tetracycline, alone or incombination with topical erythromycin, metron-idazole, or sulfur-based lotion would hasten res-olution in most patients.2, 3, 7-10 Successful treat-ment of GPD by oral doxycycline and topicalmetronidazole gel in this adult patient is encour-aging, although spontaneous improvement can-not be ruled out. In our experience, practitionersshould recognize this condition from otherfacial eruptions and therefore the patients areappropriately managed.

REFERENCES1. Gianotti F, Ermacora E, Benelli M-G, et al.: [

Particuliere dermatite periorale infantile:Observations sur cinq cas]. [French] Bull Soc FrDermatol Syphiligr 77: 341, 1970.

2. Knautz MA, Lesher JL Jr.: Childhood granuloma-tous periorificial dermatitis. Pediatr Dermatol 13:131-134, 1996.

3. Tarm K, Creel NB, Krivda SJ, et al.: Granulomatousperiorificial dermatitis. Cutis 73: 399-402, 2004.

4. Smith KW: Perioral dermatitis with histopathologicfeatures of granulomatous rosacea: successfultreatment with isotretinoin. Cutis 46: 413-415,1990.

5. Crawford GH, Pelle MT, James WD: Rosacea: I.Etiology, pathogenesis, and subtype classification.J Am Acad Dermatol 51: 327-341, 2004.

6. Hafeez ZH: Perioral dermatitis: an update. Int JDermatol 42: 514-517, 2003.

7. Urbarsch AJ, Frieden IJ, Williams ML: Extrafacialand generalized granulomatous periorificial der-matitis. Arch Dermatol 138: 1354-1358, 2002.

8. Andry P, Bodemer C, Teillac-Hamel D, et al.:Granulomatous perioral dermatitis in childhood:eight cases. Pediatr Dermatol 12: 76, 1995.

9. Frieden IJ, Prose NS, Fletcher V, et al.:Granulomatous perioral dermatitis in children.Arch Dermatol 125: 369-373, 1989.

10.Manders SM, Lucky AW: Perioral dermatitis inchildhood. J Am Acad Dermatol 27: 688-692,1992.

11. Smith EB, Powell RF, Graham JL: Periorbital der-matitis. Arch Dermatol 112: 563, 1976.

12. Georgouras K, Kocsard E: Micropapular sarcoidalfacial eruption in children. Arch Dermatol 58:433-436, 1978.

13.Skowron F, Causeret A, Pabion C, et al.:F.I.G.U.R.E.: Facial idiopathic granulomas withregressive evolution. Dermatology 201: 287-289,2000.

14.Hodak E, Trattner A, Feuerman H, et al.: Lupusmiliaris disseminatus faciei-the DNA ofMycobacterium tubeculosis is not detectable inactive lesions by polymerase chain reaction. Br JDermatol 137: 614-619, 1997.