A Multifaceted Approach to Treatment of Recalcitrant Cutaneous Periorbital Juvenile Xanthogranuloma

Abstract

Purpose: To demonstrate novel treatments for patients with high juvenile xanthogranuloma ( JXG) eyelid lesion burden.
Case Report: A 14-year-old girl was referred to the oculoplastic surgery service for management of worsening extensive bilateral eyelid and adnexal lesions in the setting of JXG. The patient underwent intra-lesional steroid injections, serial excisions, and reconstruction with skin grafts. She was subsequently treated with CO2 laser-assisted topical steroid application, which resulted in lesion regression.
Conclusion: A novel multimodal approach to treatment of severe periocular JXG, incorporating surgical debulking, skin autograft, CO2 laser, and intra-lesional steroids, can be effective for lesion control.

Keywords:

CO2 Laser, Juvenile Xanthogranuloma, Laser-assisted Steroid Delivery

References
1. Wollina U, Burgdor WHC, Haroske G. Disseminated Juvenille Xanthogranuloma. JDDG 2006;4:45–48.

2. Freyer DR, Kennedy R, Bostrom BC, Kohut G, Dehner LP. Juvenile xanthogranuloma: Forms systemic disease and their clinical implications. J Pediatr 1996;129:227–237.

3. Samara WA, Khoo CTL, Say EAT, Saktanasate J, Eagle Jr RC, Shields JA, et al. Juvenile xanthogranuloma involving the eye and ocular adnexa. Ophthalmology 2015;122:2130–2138.

4. Chang MW, Frieden IJ, Good W. The risk of intraocular juvenile xanthogranuloma: Survey of current practices and assessment of risk. J Am Acad Dermatol 1996;34:445– 449.

5. Park JH, Chun JY, Lee JH. Laser-assisted topical corticosteroid delivery for treatment of keloids. Lasers Med Sci 2017;32:601–608.

6. Cambiaghi S, Restano L, Caputo R. Juvenile xanthogranuloma associated with neurofibromatosis 1:14 patients without evidence of hematologic malignancies. Pediatr Dermatol 2004;21:97–101.

7. Zvulunov A, Barak Y, Metzker A. Juvenile xanthogranuloma, neurofibromatosis, and juvenile chronic myelogenous leukemia: World statistical analysis. Arch Dermatol 1995;131:904–908.

8. Vijapura JA, Fulbright JM. Use of radiation in treatment of central nervous system juvenile xanthogranulomatosis. Pediatr Hematol Oncol 2012;29:440–445.

9. Elner V, Mintz R, Demirci H, Hassan AS. Local corticosteroid treatment of eyelid and orbital xanthogranuloma. OPRS 2006;22:36–40.

10. Kuruvilla R, Excaravage GK, Finn AJ, Dutton JJ. Infiltrative subcutaneous juvenile xanthogranuloma of the eyelid in a neonate. OPRS 2009;25:330–332.

11. Klemke CD, Held B, Dippel E, Goerdt S. Multiple juvenile xanthogranulomas successfully treated with CO2 laser. JDDG 2007;5:30–33.

12. Vieira V, Del Pozo J, Martinez W, Veiga-Barreiro JA, Fonseca E. Necrobiotic xanthogranuloma associated with lymphoplasmacytic lymphoma. Palliative treatment with carbon dioxide laser. Eur J Dermatol 2005;15:182–185.