Adult Orbital Xanthogranuloma With Associated Adult-Onset Asthma

Abstract

: The authors report a case of adult orbital xanthogranuloma with associated adult-onset asthma in a 44-year-old man. Adult orbital xanthogranuloma was diagnosed on the basis of the clinical findings of bilateral, indurated, yellow eyelid lesions in a patient presenting with adult-onset asthma. Incisional biopsy of the eyelid lesions demonstrated a diffuse histiocytic infiltrate of the orbit and Touton giant cells without evidence of necrobiosis. Systemic evaluation failed to show evidence of bone lesions or paraproteinemia. When patients present with atypical indurated yellow eyelid lesions, a biopsy should be considered. If Touton giant cells are present, a systemic evaluation should be undertaken to rule out both Erdheim-Chester disease and necrobiotic xanthogranuloma. If no systemic findings are present, other than the possibility of adult-onset asthma, the rare entity of adult orbital xanthogranuloma should be considered.

Local Corticosteroid Treatment of Eyelid and Orbital Xanthogranuloma

“…5,6 All three types of xanthogranuloma may be associated with systemic manifestations. NXG is commonly complicated by paraproteinemia and multiple myeloma or other lymphoproliferative disorders, 1,2 whereas AXG may be associated with adult-onset asthma.…”

“…3 Erdheim-Chester disease typically involves the posterior portion of the orbit and is associated with widespread systemic disease that results in death due to infiltration of vital organs. 5,6 This disorder, 7,8 like NXG 9 and AXG, 10 has been routinely treated with combinations of systemic corticosteroids, cytotoxic agents, and radiotherapy. However, NXG and AXG tend to affect the eyelids and anterior orbital tissue.…”

Adult Orbital Xanthogranulomatous Disease

“…Clinically, this infiltration, along with accompanying lymphocytes and varying degrees of fibrosis, can replace the normal lacrimal gland architecture, causing mass effects and loss of tear production. [1][2][3][4] This review discusses the etiopathogenesis of these lesions, along with clinicopathologic presentation and associated systemic manifestations. We conclude with a discussion about current evaluation and management in patients found to have this disease.…”

“…9 With regard to specific entities, the underlying mechanism for the pathogenesis of AAPOX is understood poorly, but recent research suggests a poorly understood systemic immunologic derangement with concurrent bronchiolar and ocular adnexal dysfunction. 1,3 In addition, because the association of NXG with paraproteinemia is well-known, this fact has led to a number of hypotheses regarding the pathogenesis of this variant of AOXGD. The paraproteinemia present is an immunoglobulin (Ig)G monoclonal gammopathy that is found in at least 80% of cases of NXG.…”

Orbital inflammatory disease management

“…Although systemic steroids are typically administered for induction treatment in the form of oral prednisone 1 mg/kg/ day, recurrences frequently occur [37,39].…”