Juvenile xanthogranuloma masquerading as melanoma.
Juvenile xanthogranuloma (JXG) of the uvea is a rare disease that usually responds to systemic steroids or low-dose radiotherapy. We present an atypical case of bilateral JXG involving the entire uveal tract that presented with an aggressive phenotype. The patient was unresponsive to topical and systemic corticosteroids, cyclosporine, and maximal doses of radiation therapy. The disease was ultimately controlled with the alkylating agent chlorambucil.
Fontanilla
Fernandino A
FA
Department of Ophthalmology, University of Illinois at Chicago, Eye Infirmary, USA.
Edward
Deepak P
DP
Wong
Margaret
M
Tessler
Howard H
HH
Eagle
Ralph C
RC
Goldstein
Debra A
DA
D000893:Anti-Inflammatory Agents / Q000008:administration & dosage; D018906:Antineoplastic Agents, Alkylating / Q000008:administration & dosage*; D002648:Child; D002699:Chlorambucil / Q000008:administration & dosage*; D016572:Cyclosporine / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004351:Drug Resistance; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D008545:Melanoma / Q000175:diagnosis*; D011241:Prednisone / Q000008:administration & dosage; D012878:Skin Neoplasms / Q000175:diagnosis*; D014792:Visual Acuity; D014972:Xanthogranuloma, Juvenile / Q000175:diagnosis* / Q000188:drug therapy* / Q000532:radiotherapy
D000893:Anti-Inflammatory Agents; D018906:Antineoplastic Agents, Alkylating; D007166:Immunosuppressive Agents; D002699:Chlorambucil; D016572:Cyclosporine; D011241:Prednisone