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3-Year-Old Female With 6-Month History of Nodule on Right Cheek

Can you diagnose this case?

Petraglia A, DiMarco G, Zlotoff B.Consultant. 2018

Signs and Symptoms

A healthy 3-year-old girl presented to a dermatology clinic with a 6-month history of a nodule on her right cheek. The patient’s mother described an early increase in size of the lesion, but at the time of presentation, the size of the lesion had been stable for several months. The patient initially had presented to her pediatrician and had been prescribed mupirocin ointment, which had not improved the appearance of the lesion. The patient did not have associated fever or constitutional systems.

Physical Examination:
Examination revealed a well-appearing child with a nontender, red-purple, subcutaneous nodule overlying the right zygoma (Figure). The nodule was soft on palpation. Physical examination findings were otherwise normal, with no lymphadenopathy noted.

What are you looking at here?

Choose one to reveal diagnosis and discussion

Pilomatrixomas
Infantile nodular acne
Deep fungal infection
Idiopathic facial aseptic granuloma (IFAG)

Answer: Idiopathic facial aseptic granuloma (IFAG)

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IFAG is a relatively uncommon, benign pediatric skin lesion that occurs in children between 8 months and 13 years of age. IFAG is thought to be seen frequently in practice but is rarely reported.1 It typically presents as a solitary lesion on the cheeks or eyelids, although IFAG presenting as multiple lesions has been reported. The pathogenesis of IFAG remains unclear but may be associated with granulomatous rosacea in childhood and an increased likelihood of developing rosacea later in life, mainly ocular rosacea. Eyelid chalazion involvement also has been noted in association with IFAG.2

The differential diagnosis of IFAG includes benign tumors such as pilomatrixomas, dermoid or epidermoid cysts, infantile nodular acne, and deep fungal infections.3 IFAG, in contrast to infantile acne, in most cases is a painless nodule and lacks comedones. Pilomatrixomas can be distinguished clinically from IFAG by their firmness, tendency toward calcification, and a positive teeter-totter sign (applying pressure to one side of the lesion results in elevation of the opposite side) and/or a positive tent sign (stretching of the skin overlying the lesion reveals multiple facets/angles beneath the surface). Cultures are negative for pathogens in IFAG, in contrast to deep fungal infections. Ultrasonography, if performed, typically demonstrates a solid, well-demarcated, hypoechoic lesion without calcium deposition.4 On histopathology, IFAG shows chronic dermal lymphohistiocytic granulomas with foreign body giant cells.1 In most cases, the lesions of IFAG resolve after an average duration of 12 months. Treatment options include topical corticosteroids and oral or topical antibiotics used for rosacea (eg, oral doxycycline, topical metronidazole).5 Few cases require surgical removal.6

Outcome of the Case:
Our patient was prescribed triamcinolone ointment, 0.1%, to be applied to the affected area each night for 1 month. At the patient’s 6-month follow-up visit, the nodule had demonstrated significant regression and needed no further treatment.

REFERENCES:

  1. González Rodríguez AJ, Jordá Cuevas E. Idiopathic facial aseptic granuloma. Clin Exp Dermatol. 2015;40(3):298-300.
  2. Orion C, Sfecci A, Tisseau L, Darrieux L, Safa G. Idiopathic facial aseptic granuloma in a 13-year-old boy dramatically improved with oral doxycycline and topical metronidazole: evidence for a link with childhood rosacea. Case Rep Dermatol. 2016;8(2):197-201.
  3. Neri I, Raone B, Dondi A, Misciali C, Patrizi A. Should idiopathic facial aseptic granuloma be considered granulomatous rosacea? Report of three pediatric cases. Pediatr Dermatol. 2013;30(1):109-111.
  4. Zitelli KB, Sheil AT, Fleck R, Schwentker A, Lucky AW. Idiopathic facial aseptic granuloma: review of an evolving clinical entity. Pediatr Dermatol. 2015;32(4):e136-e139.
  5. Knöpfel N, Gómez-Zubiaur A, Noguera-Morel L, Torrelo A, Hernandez-Martin A. Ultrasound findings in idiopathic facial aseptic granuloma: case series and literature review. Pediatr Dermatol. 2018;35(3):397-400.
  6. Boralevi F, Léauté-Labrèze C, Lepreux S, et al; Groupe de Recherche Clinique en Dermatologie Pédiatrique. Idiopathic facial aseptic granuloma: a multicentre prospective study of 30 cases. Br J Dermatol. 2007;156(4):705-708.