Pediatric Annals

case challenges: dermatology 

A 9-year-old Boy With Facial Lesions

Sarah L Chamlin, MD

Abstract

A 9-year-old boy presented with a 1-month history of asymptomatic facial lesions. The lesions started around his mouth and spread to a perinasal and periocular location. His mother had been applying over-the-counter hydrocortisone twice daily for 2 weeks without noted improvement.

His medical history was unremarkable. Family history was negative for skin disease. Physical examination revealed multiple erythematous 1 to 2 mm papules with scale in a periorificial location (Figure). The remainder of the physical examination was within normal limits.

DISCUSSION

Granulomatous perioral dermatitis was initially described in the French literature by Gianotti et al. in 19701 and was later reported in the United States by Frieden et al.2 as a distinct clinical and histopathological entity. This disorder most commonly occurs in prepubertal children and is seen equally in males and females without a racial predilection.

The cutaneous lesions are erythematous or flesh colored papules distributed in a perioral, periocular, and perinasal location. The term perioral is somewhat of a misnomer; periorificial has been used to more accurately describe this eruption.3 The presence of scale and pustules in the affected areas may be noted, and some children complain of pruritus. Lesions may also occur on the neck and trunk.4 Histopathology of the papules reveals a dermal granulomatous infiltrate with surrounding lymphocytes. This granulomatous infiltrate typically predominates in perifollicular regions and follicular rapture may be noted.2 A biopsy is rarely indicated, as the diagnosis can be made by the presence of the clinical features alone.

The differential diagnosis includes acne rosacea, benign cephalic histiocytosis, allergic and irritant contact dermatitis, and sarcoidosis. Although the histologic features of acne rosacea are similar to those of perioral granulomatous dermatitis, other features of acne rosacea, including flushing, pustules, nodules, cysts and telangiectasias are not typically seen. In addition, the distribution of the lesions in acne rosacea is not primarily periorificial. Benign cephalic histiocytosis is an eruption of yellowbrown and pink papules on the forehead, cheeks and eyelids. Histologically, a dermal histiocytic infiltrate is present. Perioral granulomatous dermatitis can be distinguished from cutaneous sarcoidosis by the presence of lymphocytes and the lack of systemic findings in the former.

Treatment options include topical metronidazole cream5 or gel or systemic erythromycin (or tetracycline in children older than 8). Many children respond to topical therapy alone, but systemic therapy may be needed for persistent or severe cases. Therapeutic response often occurs slowly, and a flare of skin lesions may occur in the first weeks of therapy. Children often require treatment for several months. Topical corticosteroids have been implicated in the pathogenesis of this disorder and should be discontinued. Abrupt discontinuation of topical corticosteroids may lead to temporary worsening of the eruption. Pitted scars can be seen after resolution, but recurrences are rare.

1. Gianotti F, Ermaoora E, Benelli MG, Caputo R. Particuliere dermatite perioral infantile. Observations sur cinq cas. Bull Soc Dermatol Syphiligr. 1970;77:341.

2. Frieden IJ, Prose NS, Fletcher V, Turner ML. Granulomatous perioral dermatitis in children. Arch Dermatol. 1989;125(3):369-373.

3. Knautz MA, Lesher JL. Childhood granulomatous periorificial dermatitis. Pediatr Dermatol. 1996;13(2):131-134.

4. Hansen KK, McTigue MK, Esterly NB. Multiple facial, neck, and upper trunk papules in a black child. Childhood granulomatous perioral dermatitis with involvement of the neck and upper trunk. Arch Dermatol. 1992;128(10):1396-1397, 1399.

5. Veien NK, Munkvad JM, Nielsen AO, et al. Topical metronidazole in the treatment of perioral dermatitis. J Am Acad Dermatol. 1991;24(2 Pt 1):258-260.…

Figure. Photograph of a child's face shows perioral, perinasal, and periocular 1 to 2 mm papules.

Figure. Photograph of a child's face shows perioral, perinasal, and periocular 1 to 2 mm papules.

A 9-year-old boy presented with a 1-month history of asymptomatic facial lesions. The lesions started around his mouth and spread to a perinasal and periocular location. His mother had been applying over-the-counter hydrocortisone twice daily for 2 weeks without noted improvement.

His medical history was unremarkable. Family history was negative for skin disease. Physical examination revealed multiple erythematous 1 to 2 mm papules with scale in a periorificial location (Figure). The remainder of the physical examination was within normal limits.

DISCUSSION

Granulomatous perioral dermatitis was initially described in the French literature by Gianotti et al. in 19701 and was later reported in the United States by Frieden et al.2 as a distinct clinical and histopathological entity. This disorder most commonly occurs in prepubertal children and is seen equally in males and females without a racial predilection.

The cutaneous lesions are erythematous or flesh colored papules distributed in a perioral, periocular, and perinasal location. The term perioral is somewhat of a misnomer; periorificial has been used to more accurately describe this eruption.3 The presence of scale and pustules in the affected areas may be noted, and some children complain of pruritus. Lesions may also occur on the neck and trunk.4 Histopathology of the papules reveals a dermal granulomatous infiltrate with surrounding lymphocytes. This granulomatous infiltrate typically predominates in perifollicular regions and follicular rapture may be noted.2 A biopsy is rarely indicated, as the diagnosis can be made by the presence of the clinical features alone.

The differential diagnosis includes acne rosacea, benign cephalic histiocytosis, allergic and irritant contact dermatitis, and sarcoidosis. Although the histologic features of acne rosacea are similar to those of perioral granulomatous dermatitis, other features of acne rosacea, including flushing, pustules, nodules, cysts and telangiectasias are not typically seen. In addition, the distribution of the lesions in acne rosacea is not primarily periorificial. Benign cephalic histiocytosis is an eruption of yellowbrown and pink papules on the forehead, cheeks and eyelids. Histologically, a dermal histiocytic infiltrate is present. Perioral granulomatous dermatitis can be distinguished from cutaneous sarcoidosis by the presence of lymphocytes and the lack of systemic findings in the former.

Treatment options include topical metronidazole cream5 or gel or systemic erythromycin (or tetracycline in children older than 8). Many children respond to topical therapy alone, but systemic therapy may be needed for persistent or severe cases. Therapeutic response often occurs slowly, and a flare of skin lesions may occur in the first weeks of therapy. Children often require treatment for several months. Topical corticosteroids have been implicated in the pathogenesis of this disorder and should be discontinued. Abrupt discontinuation of topical corticosteroids may lead to temporary worsening of the eruption. Pitted scars can be seen after resolution, but recurrences are rare.

REFERENCES

1. Gianotti F, Ermaoora E, Benelli MG, Caputo R. Particuliere dermatite perioral infantile. Observations sur cinq cas. Bull Soc Dermatol Syphiligr. 1970;77:341.

2. Frieden IJ, Prose NS, Fletcher V, Turner ML. Granulomatous perioral dermatitis in children. Arch Dermatol. 1989;125(3):369-373.

3. Knautz MA, Lesher JL. Childhood granulomatous periorificial dermatitis. Pediatr Dermatol. 1996;13(2):131-134.

4. Hansen KK, McTigue MK, Esterly NB. Multiple facial, neck, and upper trunk papules in a black child. Childhood granulomatous perioral dermatitis with involvement of the neck and upper trunk. Arch Dermatol. 1992;128(10):1396-1397, 1399.

5. Veien NK, Munkvad JM, Nielsen AO, et al. Topical metronidazole in the treatment of perioral dermatitis. J Am Acad Dermatol. 1991;24(2 Pt 1):258-260.

10.3928/0090-4481-20051101-07

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