Pediatric Dermatology Consult

Make the Diagnosis

An 8-year-old African American male presented to our pediatric dermatology clinic for evaluation of a 3-month history of flesh-colored bumps on the face. According to the patient's mother, the lesions started with small pimple-like lesions around the nose and then spread to the whole face. Some lesions were crusty and somewhat itchy. He was treated with cephalexin and pimecrolimus with no improvement. The mother was very concerned because the lesions were close to the eyes and spreading.


He had no fevers, arthritis, or upper respiratory or gastrointestinal symptoms. He recently came back from a trip to Africa to visit his family. No other family members were affected. He used some new soaps, sunscreens, and moisturizers while he was in Africa.
On physical examination, the boy was in no acute distress. He had multiple flesh-colored papules on the face, especially around the eyes, nose, and mouth, where some lesions appeared crusted. There were no other skin lesions elsewhere on his body. There was no lymphadenopathy or hepatosplenomegaly.

What's your diagnosis?

Granulomatous rosacea

Childhood granulomatous periorificial dermatitis

Lupus miliaris disseminatus faciei

Cutaneous tuberculosis

Tinea

A skin biopsy of one of the lesions showed granulomatous inflammation composed of lymphocytes, macrophages, and giant cells around hair follicles with negative mycobacterium stains and fungal stains, consistent with granulomatous periorificial dermatitis. Tissue cultures from a skin biopsy for aerobic bacteria, mycobacteria, and fungus all were negative.

Dr. Catalina Matiz

The patient initially was treated with erythromycin, but after 2 weeks, he reported abdominal pain and nausea and was unable to tolerate the medication. He was switched to clarithromycin, which he took for 6 weeks with clearance of the lesions.

A year later, some of the lesions recurred. He was treated again with clarithromycin and the lesions resolved.

Childhood granulomatous periorificial dermatitis (CGPD) is a benign skin eruption that occurs in prepubertal children. It also has been called facial Afro-Caribbean childhood eruption (FACE), and it tends to occur most commonly in children of darker skin types.1CGPD is characterized by dome shape, monomorphous papules ranging from skin color to pink around the eyes, nose, and mouth, but there are some cases reported of extra facial involvement.2 The lesions usually are not symptomatic, and they are more common in boys. The cause of this condition is not known, but possible triggers could include prior exposure to topical and systemic corticosteroids, as well as exposure to certain allergens such as formaldehyde.1

In histopathology, the lesions are characterized by granulomatous infiltrates around the hair follicles and the upper dermis. The granulomas are formed of macrophages, lymphocytes, and giant cell, as were seen in our patient.3

Several conditions can look very similar to CGPD; these include sarcoidosis, lupus miliaris disseminatus faciei (LMDF), and granulomatous rosacea.

Sarcoidosis is a rare condition in children, and the lesions can be similar to the ones seen in our patient. Patients with sarcoidosis usually present with other systemic symptoms including fever, weight loss, respiratory symptoms, and fatigue; none of these were seen in our patient. Under the microscope, the lesions are characterized by “naked granulomas” instead of the inflammatory granulomas seen on our patient.

Lupus miliaris disseminatus faciei is a rare inflammatory skin condition commonly seen in young adults and is thought to be a variant of rosacea. It is characterized by skin-color to pink to yellow dome-shaped papules on the central face. Histologically, the lesions present as dermal epithelioid cell granulomas with central necrosis and surrounding lymphocytic infiltrate with multinucleate giant cells.4

Granulomatous rosacea and CGPD are considered two separate entities. Granulomatous rosacea tends to have a more chronic course, is not that common in children, and clinically presents with pustules, papules, and cysts around the eyes and cheeks.

Infectious processes like tuberculosis and fungal infections were ruled out in our patient with cultures and histopathology. Allergic contact dermatitis on the face can present with skin-color to pink papules, but they usually are very pruritic and improve with topical corticosteroids, while these medications can worsen CGPD.

CGPD can be a self-limiting condition. When mild, it can be treated with topical metronidazole, topical erythromycin, topical clindamycin solution, or pimecrolimus. Our patient failed treatment with pimecrolimus. For severe presentations, oral tetracyclines, erythromycin, and other macrolides, metronidazole, and oral isotretinoin can help clear the lesions.5

Dr. Matiz is a pediatric dermatologist at Southern California Permanente Medical Group, San Diego. She said she had no relevant financial disclosures. Email her at [email protected].

References

1. Ann Dermatol. 2011 Aug;23(3):386-8.

2. Int J Dermatol. 2007 Feb;46(2):143-5.

3. J Cutan Med Surg. 2009 Feb 28;13(2):115-8.

4. An Bras Dermatol. 2017 Nov-Dec;92(6):851-3.

5. Indian Dermatol Online J. 2018 Jan-Feb; 9(1):68-70.

Next Article: