Treatment of juvenile xanthogranuloma

Abstract

Juvenile xanthogranuloma (JXG) is generally a benign, self-limited histiocytic disorder of the skin. We report two cases of multisystem JXG presenting with clinical features more commonly seen in Langerhans cell histiocytosis (LCH), including diabetes insipidus and lytic bony lesions. Histologically, the skin lesions demonstrated a histiocytic dermal infiltrate that stained for CD-68, but S-100 and CD1a stains were negative. Treatment according to LCH-based chemotherapy regimens resulted in prompt resolution of symptoms. A literature review of multisystem JXG cases treated with chemotherapy suggests that symptomatic patients can successfully be treated with LCH-based regimens that include both corticosteroids and vinca alkaloids.

Thalidomide Therapy for Aggressive Histiocytic Lesions in the Pediatric Population

“…Initially, this therapy seemed promising, as after induction with vincristine, prednisone, cyclophosphamide, polyethylene glycol L-asparaginase, and etoposide (substituted for daunomycin), complete resolution of 18 F-FDG activity in the liver, spleen, left common iliac lymph nodes, and internal iliac lymph nodes was achieved. 18 F-FDG activity in the left calf mass, subcarinal, left external iliac nodes, and popliteal nodes persisted but was significantly diminished. With this promising initial response, protocol consolidation therapy, consisting of vincristine, polyethylene glycol L-asparaginase, etoposide, cytarabine, 6-thiogiuanine, and methotrexate, was administered.…”

Baby with neonatal systemic juvenile xanthogranuloma born within a cross-cousin marriage

“…21,22 Consequently, vinblastin, vincristine, cytarabine, methotrexate, prednisone, and 6-mercaptopurin are used for treatment. 23 Our case had a systemic disease with lung, colon, and spleen involvement; therefore, chemotherapy with prednisone and vinblastin was started. However, the child died in the 2 nd month of treatment as a result of respiratory failure due to pneumonia.…”

Intra-abdominal Xanthogranulomatoses Secondary to Postoperative Bile Leak

“…Skin lesions are usually self-limiting and may spontaneously resolve. However, adult form of the disease may not resolve spontaneously [12]. Non-resolving severe cutaneous adult form may require excision or symptomatic treatment [12].…”