Granulomatous Periorificial Dermatitis

Background

This is a rare eruption occurring in children. It is characterized by an eruption usually around the skin of the mouth, eyes, and nose. Rarely, there are extrafacial cases. Under the microscope, the pathologist will find scattered granulomas, which have a resemblance to sarcoidosis. This is a benign and self-limited condition, usually subsiding withing a few months to years. There are case reports of some patients benefitting from oral antibiotics such as tetracyclines and macrolides.

OUTLINE

Epidemiology

Gross Appearance and Clinical Variants

Histopathological Features and Variants

Differential Diagnosis

Prognosis and Treatment

Commonly Used Terms

EPIDEMIOLOGY CHARACTERIZATION

SYNONYMS Gianotti-type perioral dermatitis
Sarcoidlike granulomatous dermatitis
Facial Afro-Caribbean childhood eruption (FACE)
Granulomatous perioral dermatitis
Granulomatous periorificial dermatitis (PFD)

INCIDENCE Rare

AGE RANGE-MEDIAN Childhood

GEOGRAPHY African-American
Caribbean

ASIAN

Childhood granulomatous periorificial dermatitis in an Asian girl--a variant of sarcoid?
Antony FC, Buckley DA, Russell-Jones R.

Department of Dermatology, Ealing Hospital, Uxbridge Road, Southall, Middlesex, UK
Clin Exp Dermatol 2002 Jun;27(4):275-6 Abstract quote
Childhood granulomatous periorificial dermatitis (CGPD) is a benign, facial eruption which has previously been reported in prepubertal, Afro-Caribbean children. It is a condition of unknown aetiology, characterized by a monomorphic, papular eruption around the mouth, nose and eyes.

Typically the eruption persists for several months but resolves without scarring. The condition can usually be distinguished from sarcoidosis on clinical and histological grounds.

To our knowledge, this is the first case of CGPD occurring in an Asian child with sarcoidal histology and is unusual in its persistence for 4 years. We suggest that this case of CGPD may well be a variant of sarcoid.

GROSS APPEARANCE/
CLINICAL VARIANTS CHARACTERIZATION

GENERAL

Granulomatous perioral dermatitis in children.

Frieden IJ, Prose NS, Fletcher V, Turner ML.

Department of Dermatology, University of California, San Francisco, School of Medicine
Arch Dermatol 1989 Mar;125(3):369-73 Abstract quote
Five children, aged 3 to 11 years, developed a distinctive perioral, perinasal, and periorbital rash, consisting of tiny, closely spaced, flesh-colored "micronodules."

Histopathologic examination in all five cases revealed upper dermal and perifollicular granulomas admixed with lymphocytes. There were no associated systemic abnormalities. The lesions resolved after months to years, leaving no scars.

We propose that this condition is a form of perioral dermatitis with granulomatous histologic features, which can be distinguished from sarcoidosis and other facial eruptions in childhood both on clinical and histologic grounds.

Childhood granulomatous periorificial dermatitis.

Knautz MA, Lesher JL Jr.

Section of Dermatology, Department of Medicine, Medical College of Georgia, Augusta, USA.
Pediatr Dermatol 1996 Mar-Apr;13(2):131-4 Abstract quote
A case of childhood granulomatous periorificial dermatitis is described. This disorder occurs predominantly in prepubertal black children and is characterized by a monomorphous, papular eruption occurring around the mouth, nose, and eyes.

It is benign and self-limited. Treatment may include topical metronidazole in young patients and tetracycline in those over 8 years of age.

VARIANTS

EXTRAFACIAL

Extrafacial and generalized granulomatous periorificial dermatitis.
Urbatsch AJ, Frieden I, Williams ML, Elewski BE, Mancini AJ, Paller AS.

Department of Dermatology, University of Alabama at Birmingham, 700 18th St S, Suite 414, Birmingham, AL 35233.
Arch Dermatol 2002 Oct;138(10):1354-8
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.

HISTOLOGICAL TYPES CHARACTERIZATION

GENERAL

Micropapular sarcoidal facial eruption in a child: Gianotti-type perioral dermatitis.

Georgouras K, Kocsard E.
Acta Derm Venereol 1978;58(5):433-6 Abstract quote
The case is described of a boy aged 13 years reported with a diffuse facial micropapular eruption which had a granulomatous histology. The diagnosis is consistent with perioral dermatitis of the Gianotti type (4, 5).

It is postulated that bubble gum was the cause of the condition, the most likely ingredient being the essential oils in the gum, which may actually be selectively absorbed by the follicle to produce a perifollicular granulomatous reaction. In this article we have added yet another possible cause for perioral dermatitis.

VARIANTS

DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES

GRANULOMATOUS ROSACEA

INFECTION

FAMILIAL JUVENILE SYSTEMIC GRANULOMATOSIS (BLAU SYNDROME) Translucent skin-colored papules on trunk and extremities
Uveitis, synovitis, arthritis

Familial juvenile systemic granulomatosis (Blau's syndrome).

Scerri L, Cook LJ, Jenkins EA, Thomas AL.

Department of Dermatology, St Mary's Hospital, Portsmouth, UK
Clin Exp Dermatol 1996 Nov;21(6):445-8 Abstract quote
Blau's syndrome refers to the rare familial presentation of a sarcoid-like granulomatous disease classically involving the skin, uveal tract and joints, in the absence of pulmonary manifestations. The onset is in childhood, and the mode of inheritance is thought to be autosomal dominant.

We report a 15-year-old female and her 22-month-old daughter who presented with clinical features similar to those of Blau's syndrome. In addition to the skin, eye and joint disease, the mother also developed neurological involvement. In both patients long-term systemic corticosteroids were required to control the disease.

Familial granulomatous arthritis (Blau syndrome) with granulomatous renal lesions.

Ting SS, Ziegler J, Fischer E.

Department of Immunology/Allergy, Sydney Children's Hospital, Nepean Hospital, NSW, Australia.
J Pediatr 1998 Sep;133(3):450-2 Abstract quote
Blau syndrome is a granulomatous disease of the skin, eyes, and joints, usually without visceral involvement. It is inherited in a autosomal dominant manner. The Blau susceptibility locus has been mapped to chromosome 16 p 12-q21. A recent report has added liver granulomata.

We describe a family with Blau syndrome in whom 1 member had renal interstitial granulomata.

The Blau syndrome gene is not a major risk factor for sarcoidosis.

Rybicki BA, Maliarik MJ, Bock CH, Elston RC, Baughman RP, Kimani AP, Sheffer RG, Chen KM, Major M, Popovich J Jr, Iannuzzi MC.

Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit, MI.
Sarcoidosis Vasc Diffuse Lung Dis 1999 Sep;16(2):203-8 Abstract quote
BACKGROUND: A genetic predisposition to sarcoidosis has long been postulated, although no specific susceptibility genes are known. Candidate genes for the two granulomatous inflammatory disorders with clinical similarities to sarcoidosis, Blau syndrome and Crohn's disease, have been localized to a 40 centimorgan region spanning the chromosome 16 centromere.

PATIENTS AND METHODS: Using a sample of 35 African-American sibling pairs, who both had clinically confirmed sarcoidosis, we tested for genetic linkage between the 16p12-q21 interval (the likely location of the Blau syndrome gene) and sarcoidosis.

RESULTS: We found no evidence for linkage to any of the eight markers we tested in the 16p12-q21 interval. Ninety percent of the 16p12-q21 region had a LOD score < -2 for a dominant gene conferring a relative risk of 3 or greater for sarcoidosis. One hundred percent of the region had a LOD score < -2 for a dominant gene with a relative risk of 3.5 or greater or recessive gene with relative risk of 2.5 or greater. Based on simulation results we could not exclude a dominant gene with relative risk < 5 at the 0.05 significance level, nor a recessive gene with relative risk < 3, over the entire 16p12-q21 interval.

CONCLUSIONS: While the clinical similarities between Blau Syndrome and sarcoidosis suggest genetic homogeneity between the disorders, we found no evidence for linkage of sarcoidosis to the Blau syndrome locus. Our exclusion results suggest that the Blau Syndrome gene does not have a major effect on sarcoidosis susceptibility.

PROGNOSIS AND TREATMENT CHARACTERIZATION

PROGNOSTIC FACTORS

TREATMENT

ISOTRETINOIN

Perioral dermatitis with histopathologic features of granulomatous rosacea: successful treatment with isotretinoin.

Smith KW.

Anderson Skin and Cancer Clinic, South Carolina 29621
Cutis 1990 Nov;46(5):413-5 Abstract quote
A young woman with a persistent eruption of small granulomas about the mouth and chin was studied and granulomatous perioral dermatitis was diagnosed. Some authorities consider that perioral dermatitis is a circumscribed variant of rosacea; the findings in this case tend to show a relationship between the two conditions.

Once conventional therapies failed to result in a response, the eruption cleared, leaving pitted, atrophic scarring after a twenty week course of isotretinoin treatment.

TETRACYCLINE

Sarcoid-like granulomatous periocular dermatitis treated with tetracycline.

Falk ES.
Acta Derm Venereol 1985;65(3):270-2 Abstract quote
Two cases of periocular micropapular dermatitis with sarcoid-like granulomatous histology are reported. After 8-12 weeks treatment with tetracycline the cutaneous eruptions of both patients cleared.

The condition is equivalent to perioral dermatitis.

Weedon D. Weedon's Skin Pathology. Second Edition. Churchill Livingstone. 2002.
Fitzpatrick's Dermatology in General Medicine. 5th Edition. McGraw-Hill. 1999.

Commonly Used Terms

Skin Rash

Last Updated 10/28/2002

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
GENERAL
Granulomatous perioral dermatitis in children. Frieden IJ, Prose NS, Fletcher V, Turner ML. Department of Dermatology, University of California, San Francisco, School of Medicine	Arch Dermatol 1989 Mar;125(3):369-73 Abstract quote Five children, aged 3 to 11 years, developed a distinctive perioral, perinasal, and periorbital rash, consisting of tiny, closely spaced, flesh-colored "micronodules." Histopathologic examination in all five cases revealed upper dermal and perifollicular granulomas admixed with lymphocytes. There were no associated systemic abnormalities. The lesions resolved after months to years, leaving no scars. We propose that this condition is a form of perioral dermatitis with granulomatous histologic features, which can be distinguished from sarcoidosis and other facial eruptions in childhood both on clinical and histologic grounds.
Childhood granulomatous periorificial dermatitis. Knautz MA, Lesher JL Jr. Section of Dermatology, Department of Medicine, Medical College of Georgia, Augusta, USA.	Pediatr Dermatol 1996 Mar-Apr;13(2):131-4 Abstract quote A case of childhood granulomatous periorificial dermatitis is described. This disorder occurs predominantly in prepubertal black children and is characterized by a monomorphous, papular eruption occurring around the mouth, nose, and eyes. It is benign and self-limited. Treatment may include topical metronidazole in young patients and tetracycline in those over 8 years of age.
VARIANTS
EXTRAFACIAL
Extrafacial and generalized granulomatous periorificial dermatitis. Urbatsch AJ, Frieden I, Williams ML, Elewski BE, Mancini AJ, Paller AS. Department of Dermatology, University of Alabama at Birmingham, 700 18th St S, Suite 414, Birmingham, AL 35233.	Arch Dermatol 2002 Oct;138(10):1354-8 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.

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
GRANULOMATOUS ROSACEA
INFECTION
FAMILIAL JUVENILE SYSTEMIC GRANULOMATOSIS (BLAU SYNDROME)	Translucent skin-colored papules on trunk and extremities Uveitis, synovitis, arthritis
Familial juvenile systemic granulomatosis (Blau's syndrome). Scerri L, Cook LJ, Jenkins EA, Thomas AL. Department of Dermatology, St Mary's Hospital, Portsmouth, UK	Clin Exp Dermatol 1996 Nov;21(6):445-8 Abstract quote Blau's syndrome refers to the rare familial presentation of a sarcoid-like granulomatous disease classically involving the skin, uveal tract and joints, in the absence of pulmonary manifestations. The onset is in childhood, and the mode of inheritance is thought to be autosomal dominant. We report a 15-year-old female and her 22-month-old daughter who presented with clinical features similar to those of Blau's syndrome. In addition to the skin, eye and joint disease, the mother also developed neurological involvement. In both patients long-term systemic corticosteroids were required to control the disease.
Familial granulomatous arthritis (Blau syndrome) with granulomatous renal lesions. Ting SS, Ziegler J, Fischer E. Department of Immunology/Allergy, Sydney Children's Hospital, Nepean Hospital, NSW, Australia.	J Pediatr 1998 Sep;133(3):450-2 Abstract quote Blau syndrome is a granulomatous disease of the skin, eyes, and joints, usually without visceral involvement. It is inherited in a autosomal dominant manner. The Blau susceptibility locus has been mapped to chromosome 16 p 12-q21. A recent report has added liver granulomata. We describe a family with Blau syndrome in whom 1 member had renal interstitial granulomata.
The Blau syndrome gene is not a major risk factor for sarcoidosis. Rybicki BA, Maliarik MJ, Bock CH, Elston RC, Baughman RP, Kimani AP, Sheffer RG, Chen KM, Major M, Popovich J Jr, Iannuzzi MC. Department of Biostatistics and Research Epidemiology, Henry Ford Health System, Detroit, MI.	Sarcoidosis Vasc Diffuse Lung Dis 1999 Sep;16(2):203-8 Abstract quote BACKGROUND: A genetic predisposition to sarcoidosis has long been postulated, although no specific susceptibility genes are known. Candidate genes for the two granulomatous inflammatory disorders with clinical similarities to sarcoidosis, Blau syndrome and Crohn's disease, have been localized to a 40 centimorgan region spanning the chromosome 16 centromere. PATIENTS AND METHODS: Using a sample of 35 African-American sibling pairs, who both had clinically confirmed sarcoidosis, we tested for genetic linkage between the 16p12-q21 interval (the likely location of the Blau syndrome gene) and sarcoidosis. RESULTS: We found no evidence for linkage to any of the eight markers we tested in the 16p12-q21 interval. Ninety percent of the 16p12-q21 region had a LOD score < -2 for a dominant gene conferring a relative risk of 3 or greater for sarcoidosis. One hundred percent of the region had a LOD score < -2 for a dominant gene with a relative risk of 3.5 or greater or recessive gene with relative risk of 2.5 or greater. Based on simulation results we could not exclude a dominant gene with relative risk < 5 at the 0.05 significance level, nor a recessive gene with relative risk < 3, over the entire 16p12-q21 interval. CONCLUSIONS: While the clinical similarities between Blau Syndrome and sarcoidosis suggest genetic homogeneity between the disorders, we found no evidence for linkage of sarcoidosis to the Blau syndrome locus. Our exclusion results suggest that the Blau Syndrome gene does not have a major effect on sarcoidosis susceptibility.

Epidemiology
Gross Appearance and Clinical Variants
Histopathological Features and Variants
Differential Diagnosis
Prognosis and Treatment
Commonly Used Terms

EPIDEMIOLOGY	CHARACTERIZATION
SYNONYMS	Gianotti-type perioral dermatitis Sarcoidlike granulomatous dermatitis Facial Afro-Caribbean childhood eruption (FACE) Granulomatous perioral dermatitis Granulomatous periorificial dermatitis (PFD)
INCIDENCE	Rare
AGE RANGE-MEDIAN	Childhood
GEOGRAPHY	African-American Caribbean
ASIAN
Childhood granulomatous periorificial dermatitis in an Asian girl--a variant of sarcoid? Antony FC, Buckley DA, Russell-Jones R. Department of Dermatology, Ealing Hospital, Uxbridge Road, Southall, Middlesex, UK	Clin Exp Dermatol 2002 Jun;27(4):275-6 Abstract quote Childhood granulomatous periorificial dermatitis (CGPD) is a benign, facial eruption which has previously been reported in prepubertal, Afro-Caribbean children. It is a condition of unknown aetiology, characterized by a monomorphic, papular eruption around the mouth, nose and eyes. Typically the eruption persists for several months but resolves without scarring. The condition can usually be distinguished from sarcoidosis on clinical and histological grounds. To our knowledge, this is the first case of CGPD occurring in an Asian child with sarcoidal histology and is unusual in its persistence for 4 years. We suggest that this case of CGPD may well be a variant of sarcoid.

HISTOLOGICAL TYPES	CHARACTERIZATION
GENERAL
Micropapular sarcoidal facial eruption in a child: Gianotti-type perioral dermatitis. Georgouras K, Kocsard E.	Acta Derm Venereol 1978;58(5):433-6 Abstract quote The case is described of a boy aged 13 years reported with a diffuse facial micropapular eruption which had a granulomatous histology. The diagnosis is consistent with perioral dermatitis of the Gianotti type (4, 5). It is postulated that bubble gum was the cause of the condition, the most likely ingredient being the essential oils in the gum, which may actually be selectively absorbed by the follicle to produce a perifollicular granulomatous reaction. In this article we have added yet another possible cause for perioral dermatitis.
VARIANTS

PROGNOSIS AND TREATMENT	CHARACTERIZATION
PROGNOSTIC FACTORS
TREATMENT
ISOTRETINOIN
Perioral dermatitis with histopathologic features of granulomatous rosacea: successful treatment with isotretinoin. Smith KW. Anderson Skin and Cancer Clinic, South Carolina 29621	Cutis 1990 Nov;46(5):413-5 Abstract quote A young woman with a persistent eruption of small granulomas about the mouth and chin was studied and granulomatous perioral dermatitis was diagnosed. Some authorities consider that perioral dermatitis is a circumscribed variant of rosacea; the findings in this case tend to show a relationship between the two conditions. Once conventional therapies failed to result in a response, the eruption cleared, leaving pitted, atrophic scarring after a twenty week course of isotretinoin treatment.
TETRACYCLINE
Sarcoid-like granulomatous periocular dermatitis treated with tetracycline. Falk ES.	Acta Derm Venereol 1985;65(3):270-2 Abstract quote Two cases of periocular micropapular dermatitis with sarcoid-like granulomatous histology are reported. After 8-12 weeks treatment with tetracycline the cutaneous eruptions of both patients cleared. The condition is equivalent to perioral dermatitis.