Extensive juvenile xanthogranuloma with autoimmune lymphoproliferative syndrome: report of a case and review
Keywords:
Juvenile xanthogranuloma, Autoimmune lymphoproliferative syndrome, RituximabAbstract
Juvenile xanthogranuloma (JXG) is an unusual disorder belonging to the group of non-Langerhans cell histiocytosis. Most clinical presentation affects only the skin, but extracutaneous manifestations are not uncommon such as ocular, lung and hematologic involvement. We reported a 6 months old girl presented with extensive lesions of JXG concomitant with thrombocytopenia and hemolytic anemia which finally diagnosed with autoimmune lymphoproliferative syndrome (ALPS). She was treated with intravenous immunoglobulin (IVIG), rituximab and mycophenolate mofetil (MMF). After 6 months of treatment, thrombocytopenia and anemia gradually returned to normal. The spontaneous regression of some skin lesions of JXG were observed after 1 year old. Clinical of ALPS were in remission. To our knowledge, there is no report in the English literature regarding the association between JXG and ALPS.
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เนื้อหาและข้อมูลในบทความที่ลงตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นข้อคิดเห็นและความรับผิดชอบของผู้เขียนบทความโดยตรงซึ่งกองบรรณาธิการวารสาร ไม่จำเป็นต้องเห็นด้วย หรือร่วมรับผิดชอบใดๆ
บทความ ข้อมูล เนื้อหา รูปภาพ ฯลฯ ที่ได้รับการตีพิมพ์ในวารสารโรคผิวหนัง ถือเป็นลิขสิทธิ์ของวารสารฯ หากบุคคลหรือหน่วยงานใดต้องการนำทั้งหมดหรือส่วนหนึ่งส่วนใดไปเผยแพร่ต่อหรือเพื่อกระทำการใดๆ จะต้องได้รับอนุญาตเป็นลายลักอักษรจากบรรณาธิการวารสารโรคผิวหนังก่อนเท่านั้น
