Case for diagnosis

Abstract

Childhood Granulomatous Periorificial Dermatitis is an acneiform facial rash that affects the periorificial area in children. The clinical aspectare asymptomatic 1-3 mm papules of, monomorphic, erythematous or hypopigmented in periorificial areas - mouth, nose and eyes. It's a benign and self-limited disease that heals spontaneously without scarring and specific therapy. Differential diagnoses include perioral dermatitis, granulomatous-rosacea, sarcoidosis, and lupus miliaris disseminatus faciei. We present the case of a 4-year-old boy, presenting papules in periorificials areas. Due to its low incidence and low number of publications we report the present case.

CASE REPORT

A 4-year-old blackmale patient, who was born and raised in Santos - SP, presented with normochromic and asymptomatic papular eruption on the face for two years. There was no personal or family history of other skin conditions including acne, atopic or contact dermatitis. He had not done previously topical or systemic treatment. The morphology of the lesions was characterized by numerous monomorphic papules affecting the perioral region predominantly and less commonly in peri-ocular e peri-nasal regions. (Figures 1 and 2)

FIGURE 1.

Patient with papular eruption normochromic areas periorificial

FIGURE 2.

Detail of a papular eruption in the perioral region normochromic

Histopathologic examination of a perioral papule showed lymphohistiocytic inflammatory infiltrate affecting the superficial dermis and perifollicular areas with multinucleated giant cells forming multiple granulomas without central caseous necrosis over the dermis extension. The special staining for fungi (Grocott) and Mycobacteria were negative (Figure 3).

FIGURE 3.

lymphohistiocytic inflammatory infiltrate affecting peri-follicular, with multinucleated giant cells, affecting the periphery of the follicle. HE x100

Initial therapy with topical clindamycin 1% associated with benzoyl peroxide 5% at night for 4 months showed gradual improvement. It remains in service.

DISCUSSION

The Childhood granulomatous periorificial dermatitis (CGPD) is a benign acneiform facial rash that affects children and is clinically similar to rosacea and perioral dermatitis of childhood.1 The current terminology originates in 1996 (Knautz and Lesher), although other nomenclatures have been proposed, such as Afro-Caribbean child facial rash (FACE), reflecting its incidence in black children.² It is more common in the cephalic segment of healthy prepuberal, through asymptomatic papules affecting periorificial areas (mouth, nose and eyes), 1-3 mm, monomorphic, erythematous or hypopigmented, or even yellow-red, and typical characteristic by absence of pustules. ³ The age range is between 3-12 years.^4,5 It affects mostly black patients, but there are reports of cases involving Caucasian patients.⁶ The absence of pustules and the presence of yellowish or erythematous papules associated with an infiltration of perifollicular granulomas differentiates CGPD from perioral dermatitis. ⁷

The etiology is controversial and in some cases the anamnesis presents use of topical medication and there are reports of reaction to bubble gum, formaldehyde, cosmetics and antiseptic solutions for oral hygiene. ^6,7 The use of topical corticosteroids may be responsible for the appearance of the lesions.⁷

Histopathologic examination shows perifollicular granulomas composed of epithelioid macrophages, lymphocytes and giant cells. In some sections there is more diffuse lymphocytic infiltrate with wellformed noncaseating granulomas. The epidermis may show moderate spongiosis. The results of special stains and cultures for mycobacteria and fungi are always negative.²

The CGPD must be clinically differentiated from sarcoidosis ( few cases in children) and when present, there is systemic involvement and general symptoms (fever, malaise, cough and dyspnea). Histologically, it is distinguished by the "naked granulomas" when there is an absence of inflammatory cells involving the granuloma.⁴

Granulomatous rosacea affects the central area of the face and is characterized by erythema, telangiectasia, pustules, flushing and edema. It is more common in women between 30-50 years-old.⁸

Disseminated lupus miliary of the face is characterized by chronic papular eruption, occurs primarily in the central area of the face. The lesions are usually red or yellowish, acuminate, with a predilection for the eyelid and is most common in adults. Histologically there are granulomas with central caseous necrosis, but there are some reported cases without necrosis. The definitive diagnosis is by histology, clinical presentation and resolution with residual scarring, which does not occur in CGPD.²

The treatment consists in suspension of any topical corticosteroids and there are reports of improvement with oral tetracycline and metronidazole and topical erythromycin. It is important to explain the patient and his family that it is a benign disease and has self-limited evolution, healing without scar.³