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Features Departments Information |
 Sarah L. Chamlin, MD |
Dermatology quiz Sarah L. Chamlin, MD aFall 2001  A 9-year-old male presents with a mildly pruritic facial eruption for approximately 4 months' duration. Prior to presentation to dermatology, he was treated with multiple topical mid-potency corticosteroids, metronidazole 0.75% cream, erythromycin 2% gel, tacrolimus 0.1% ointment, and a 5-day course of systemic corticosteroids. The eruption worsened despite these therapies. Medical history is positive for febrile seizures as an infant. Physical examination reveals multiple periocular, perioral, and perinasal erythematous papules and a few pustules. These papules extend onto the lower conjunctivae of both eyes. The remainder of his physical examination is completely normal. The diagnosis is: The most appropriate therapy includes: This eruption: Answers: D, D, and A, respectively. Diagnosis: Granulomatous perioral dermatitis Discussion: The patient has granulomatous perioral dermatitis. his disorder was initially described in the French literature by Gianotti et al in 1970.[1] Later, Frieden et al reported 5 children with similar eruptions and described distinctive clinical and histopathological features.[2] Subsequently, these features have been described in many additional reports.[3-5] This disorder occurs most commonly in prepubertal children, with a predominance of African-American children reported. Clinically, the lesions are erythematous and flesh-colored papules distributed in the perioral, periocular, and perinasal regions. The term perioral is somewhat of a misnomer, and periorificial has been used to more accurately describe this dermatitis.[5] The presence of scale and pustules in the affected areas may be noted. Some children complain of pruritus. Histopathology of the papules reveals an upper dermal granulomatous infiltrate with surrounding lymphocytes. This granulomatous infiltrate typically predominates in the perifollicular regions, and follicular rupture may be noted.[2] A biopsy is not usually indicated in the evaluation, as the diagnosis can be made by the presence of the clinical features alone. The differential diagnosis in this patient includes perioral dermatitis, acne rosacea, benign cephalic histiocytosis, and sarcoidosis. Perioral dermatitis is characterized by erythematous papules primarily in a perioral distribution. It occurs most commonly in adult women, but can occur in children and is often linked with the use of topical corticosteroids. Although the histological features of acne rosacea are similar to the features seen in perioral granulomatous dermatitis, other features of acne rosacea, including flushing, pustules, nodules, cysts, and telangiectasias are not typical. The distribution of the lesions in acne rosacea is not primarily periorificial. Benign cephalic histiocytosis is an eruption of yellow-brown 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 lack of systemic findings in the former. Treatment options for perioral granulomatous dermatitis include topical metronidazole and/or systemic erythromycin (or tetracycline/doxycycline in children older than 8 years of age). Although some children respond to topical therapy alone, more often systemic therapy is required. Improvement with topical and/or systemic therapies occurs slowly, and most often children will require treatment for several months. In addition, the use of corticosteroids should be discontinued, as their use worsens this eruption. Abrupt discontinuation of corticosteroids often leads to worsening of the skin lesions, and parents should be reassured that this is expected and temporary. REFERENCES 1. Gianotti F, Ermacora E, Benelli M-G, 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;125:369–373. 3. Williams HC, Ashworth J, Pembroke AC, Breathnach SM. FACE—facial Afro-Caribbean childhood eruption. Clin Exp Dermatol 1990;15:163–166. 4. Hansen KK, McTigue MK, Esterly NB. Multiple facial, neck, and upper trunk papules in a black child: granulomatous perioral dermatitis with involvement of the neck and upper trunk. Arch Dermatol 1992;128:1395. 5. Knautz MA, Lesher JL. Childhood granulomatous periorificial dermatitis. Pediatr Dermatol 1996;13:131–134. |