A toddler with facial nodules: A case of idiopathic facial aseptic granuloma
- Author(s): Martinez-Diaz, Gabriel J
- Kim, Jinah
- Bruckner, Anna L
- et al.
Published Web Locationhttps://doi.org/10.5070/D3806360j4
A toddler with facial nodules: A case of idiopathic facial aseptic granuloma1. Stanford University School of Medicine
Gabriel J Martinez-Diaz BS1, Jinah Kim MD PhD2, Anna L Bruckner MD3
Dermatology Online Journal 16 (1): 9
2. Departments of Pathology and Dermatology, Stanford University
3. Departments of Dermatology and Pediatrics, Stanford University School of Medicine
We describe the case of a 3-year-old boy who presented with several asymptomatic facial nodules present for six months. A skin biopsy obtained from the nodules showed a moderately well-defined granuloma in the superficial and deep dermis. A squamous epithelial lined cyst, extravasated keratin, or shadow cells were not identified. Bartonella henselae titers and the Coccidioidomycosis immitis immunodiffusion test were negative; a Tuberculin Skin Test was non-reactive. Fite, Periodic acid-Schiff (PAS) and Gomori-Grocott methenamine silver (GMS) stains failed to identify microorganisms. In addition, tissue cultures for bacteria, fungus, and acid fast bacilli were negative. In light of the clinical findings, histology, and negative cultures, a diagnosis of idiopathic facial aseptic granuloma (IFAG) was made. After the biopsy, the child was treated with erythromycin and clarithromycin, each for one month, and the lesions slowly improved.
A 3-year-old boy presented for evaluation of multiple facial nodules that had been present for six months. After their initial appearance, the lesions sporadically fluctuated in size but never resolved completely. They were largely asymptomatic, although the child remarked that the lesions were painful at times. The nodules had been treated with topical Nystatin with no improvement. He denied prior health problems. He was born and raised in an agricultural region of California and had not travelled outside of the United States. He lived in a trailer home with feral cats in the neighborhood.
|Figure 1||Figure 2|
Physical examination showed a well-appearing toddler with two dermal, erythematous-to-violaceous nodules on each cheek (Figures 1 and 2). They ranged from 4 mm to 1.5 cm in size, were moderately firm, and were not fluctuant. There was no pre-auricular or cervical lymphadenopathy. The remainder of his examination was normal.
An ultrasound showed that the lesions were homogenous, hypoechoic and solid. They did not improve after treatment with cephalexin for 10 days. Bartonella henselae titers and a Coccidioidomycosis immitis immunodiffusion test were negative; a Tuberculin Skin Test was non-reactive. The nodules were persistent and caused significant parental anxiety. Thus, two months after his initial presentation, biopsies of the right lateral cheek lesion were obtained (Figure 3).
Microscopic findings and clinical course
Histologic examination revealed a moderately well-defined granuloma in the superficial and deep dermis (Figure 3). A peripheral rim of lymphocytes, neutrophils, and eosinophils is seen. In addition, histiocytes and foreign body-type giant cells were abundant in the center of the lesion (see inset of Figure 3). The differential diagnosis for this suppurative granuloma included infections and foreign body reaction . A squamous epithelial-lined cyst, extravasated keratin, or shadow cells were not identified. Fite, Periodic acid-Schiff (PAS) and Gomori-Grocott methenamine silver (GMS) stains failed to identify microorganisms. In addition, tissue cultures for bacteria, fungus, and acid fast bacilli were negative. In light of the clinical findings, histology, and negative cultures, a diagnosis of idiopathic facial aseptic granuloma (IFAG) was made. After the biopsy, the child was treated with erythromycin and clarithromycin, each for one month, and the lesions slowly improved.
Idiopathic facial aseptic granuloma is a pediatric entity characterized by painless red to violaceous nodules, frequently located on cheeks and eyelids. It was first described in 1999  by a group of French dermatologists as pyodermite froide and then renamed in 2001 by Roul et al . A prospective study of 30 cases of children with IFAG reported the average lesion duration to be 11 months, with lesions typically presenting centrally on the cheeks. A prolonged course is usual, but spontaneous healing evenutally occurs. Ultrasound studies have shown that the lesions are solid and cultures for bacteria and mycobacteria, and antibody tests for Bartonella henselae and Afipia felis are negative . Lesions may be noticed after mild trauma or an insect bite and often heal spontaneously without leaving a permanent scar . Local or systemic antibiotics are generally ineffective. In this case, it is unclear if treatment with antibiotics or simply "tincture of time" led to resolution of the lesions. Although the exact etiology is not clear, Boralevi et al.  postulated that IFAG may be related to a granulomatous process surrounding an embryological residue or a manifestation of granulomatous rosacea of childhood.
This case is presented to emphasize the importance of considering IFAG in the differential diagnosis of acquired, dermal, facial nodules in children. The characteristic appearance and location of the lesions in the absence of adenopathy and constitutional symptoms should make the clinical diagnosis straightforward and prevent unnecessary interventions. Counseling on the benign nature of the disorder and close follow up until resolution are advised.
References1. Weedon, D. The Granulomatous Reaction Patterns. Skin Pathology 2008:194-213.
2. Léauté-Labrèze C MJ, Taïeb (ed). A. Dermatoses bactériennes. 3rd edn ed. Paris: Masson; 1999. 114-21.
3. Roul S, Léauté-Labrèze C, Boralevi F, Bioulac-Sage P, Maleville J, Taieb A. Idiopathic aseptic facial granuloma (pyodermite froide du visage): a pediatric entity? Arch Dermatol. 2001;137(9):1253-5. [PubMed]
4. Boralevi F, Léauté-Labrèze C, Lepreux S, et al. Idiopathic facial aseptic granuloma: a multicentre prospective study of 30 cases. Br J Dermatol. 2007;156(4):705-8. [PubMed]
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