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Periocular Necrobiotic Xanthogranuloma Response to Cladribine

Agrawal, Sahil M.D.*,†; Pushker, Neelam M.D.*,†; Modaboyina, Sujeeth M.D.*,†; Das, Deepsekhar M.D.*,†

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Ophthalmic Plastic and Reconstructive Surgery: March/April 2022 - Volume 38 - Issue 2 - p e64
doi: 10.1097/IOP.0000000000002037
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A 42-year-old woman presented to the Ophthalmology department with bilateral large nodular lesions around her eyes for the past 2 years (panel A). On examination, yellow to skin colored, ill as well as well-defined multiple papules and nodules were noted around the periorbital area, forehead, and neck. Many papules had coalesced to form large plaques. Blood investigations including electrophoresis were performed to rule out paraproteinemia. A biopsy from the periocular lesion revealed necrobiotic granulomatous inflammation, atypical giant cells, cholesterol clefts, plasma cells, and minimal fibrosis. A diagnosis of xanthogranuloma necrobiotica was made. Intralesional steroids were injected, and the patient was started on oral steroids. However, there was no response. Eventually, 4 cycles of intravenous cladribine were administered with routine monitoring of liver function, kidney function, and other blood parameters. A marked reduction in the size of the lesions were noted. (Panel B) There was no obvious systemic adverse reaction of the drug and the lesions did not flare up once the chemotherapy was halted.

Necrobiotic xanthogranuloma is a rare, chronic and gradually progressive disease characterized by yellowish plaque lesions typically involving the periorbital area and unlike other orbital xanthogranulomas, having a high propensity to ulcerative and fibrose. The clinical condition principally involves the skin and subcutaneous tissue and is known to be associated with characteristic dermatopathologic findings, monoclonal gammopathy, and less commonly malignant tumors. Medical management including chemotherapy with alkylating agents and antimetabolites as well as administration of intralesional, topical and systemic steroids has yielded varied results. Although multiple other modes of treatment have also been advocated in the management of xanthogranuloma necrobiotica ranging from, radiation therapy, plasmapheresis, surgical excision, or a combination of them, there still remains a dispute regarding the definite management. In the recent times, cladribine has shown encouraging results in the management of xanthoma disseminatum, with that knowledge in background the authors used the drug in the management of xanthogranuloma necrobiotica.

A, Clinical picture of the patient at presentation showing bilateral huge periocular nodular lesions. B, Clinical picture of the patient after 4 cycles of Cladribine showing reduction in size of the lesions.
© 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.