Periocular Xanthogranuloma Research Articles

Azathioprine and prednisone combination treatment for adult periocular and orbital xanthogranulomatous disease

To report the authors' experience with azathioprine and prednisone combination for adult periocular and orbital xanthogranulomatous disease. We identified 13 adults with histology-proven periocular or orbital xanthogranuloma in two tertiary referral orbital centres from 1984 to 2008. Patient records were reviewed and data collected on orbital localization, immune dysfunction, applied treatment and outcome. Five patients with periocular or orbital xanthogranulomatous disease were fully treated with prednisone and azathioprine combination, which resulted in stabilization in two and regression in three. Two other patients had to discontinue azathioprine because of side-effects. Of the non-fully treated prednisone/azathioprine patients, four out of eight progressed. In adult periocular and orbital xanthogranuloma, combined treatment with prednisone and azathioprine yields adequate immunosuppression, often for a prolonged period of time.

Periocular xanthogranuloma: A forgotten entity?

Periocular xanthogranulomatous diseases are a rare group of disorders which are characterized by a predilection to affect the orbit and ocular adnexa and special histopathological features, in particular infiltrates comprising non-Langerhans-derived foamy histiocytes and Touton giant cells. The differential diagnosis is difficult and occasionally definite diagnosis cannot be established even after clinical and histopathological findings are taken together. We describe a case of a middle-aged man who presented with a 10-year history of voluminous eyelid swelling with concomitant late-onset atopic manifestations, namely bronchial asthma and allergic rhinitis with nasal polyps. After thorough clinical and laboratory investigation, including a biopsy of the eyelid, we classified the patient's disease to a rare entity that has been relatively recently described: periocular xanthogranuloma associated with adult-onset asthma. In a review of the literature, no prospective trials concerning the treatment of this disease were found. The literature mainly contained case reports and case series in which corticosteroids and chemotherapy with alkylating agents have been reported to be beneficial. We treated our patient with a combination of oral corticosteroids and cyclophosphamide pulses and we observed substantial regression of the eyelid masses together with a normalization of systemic immunologic abnormalities.

Adult orbital xanthogranulomatous disease: review of the literature.

This article provides an overview of the pathologic features of adult orbital xanthogranulomatous disease, a rare heterogeneous group of disorders that includes 4 clinical syndromes: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease. The diagnosis is made by biopsy of the lesion, demonstrating tissue infiltration by the hallmarks of xanthoma cells and Touton giant cells. The differential diagnosis is broad, including syndromes within the adult xanthogranulomatous disease category as well as other entities involving the eyelid and the orbital tissues. Because of its rarity and sometimes close similarity to other disease entities, it is often misdiagnosed initially. This article focuses on the morphology and immunohistochemical patterns in diagnosis of adult orbital xanthogranulomatous disease with emphasis on adult-onset asthma and periocular xanthogranuloma in particular, its clinical features and associated systemic manifestations in differential diagnosis, as well as the current management strategy.

Adult Orbital Xanthogranulomatous Disease: Review of the Literature

This article provides an overview of the pathologic features of adult orbital xanthogranulomatous disease, a rare heterogeneous group of disorders that includes 4 clinical syndromes: adult-onset xanthogranuloma, necrobiotic xanthogranuloma, adult-onset asthma and periocular xanthogranuloma, and Erdheim-Chester disease. The diagnosis is made by biopsy of the lesion, demonstrating tissue infiltration by the hallmarks of xanthoma cells and Touton giant cells. The differential diagnosis is broad, including syndromes within the adult xanthogranulomatous disease category as well as other entities involving the eyelid and the orbital tissues. Because of its rarity and sometimes close similarity to other disease entities, it is often misdiagnosed initially. This article focuses on the morphology and immunohistochemical patterns in diagnosis of adult orbital xanthogranulomatous disease with emphasis on adult-onset asthma and periocular xanthogranuloma in particular, its clinical features and associated systemic manifestations in differential diagnosis, as well as the current management strategy.

Multiple periocular adult onset xanthogranulomas in a patient with chronic lymphocytic leukaemia

Adult and juvenile forms of cutaneous xanthogranuloma share many clinical and histopathological features. Unlike the juvenile variant, systemic disorders including haematological malignancies have only rarely been reported in association with adult xanthogranuloma. We report a case of multiple periocular xanthogranulomas arising in a 43 year old man with chronic lymphocytic leukaemia. This case supports the emerging hypothesis that both juvenile and adult xanthogranulomas may share an association with an underlying haematological malignancy. Also highlighted is the fact that some patients with periocular cutaneous xanthogranulomas of adult onset can have co-existing orbital xanthogranulomatous disease and appropriate clinical and radiological investigations may be needed for accurate diagnosis.

Clinical Manifestations and Treatment of Adult‐Onset Asthma and Periocular Xanthogranuloma

Adult-onset asthma and periocular xanthogranuloma is an uncommon and recently described disease. Little is known about the condition because only a few case reports and series are available. To describe the clinical manifestations, lung physiology, and response to systemic treatment of three patients with adult-onset asthma and periocular xanthogranuloma, followed by a review of the literature. Three men, with an age at diagnosis ranging from 48 to 51 years, presented with right periorbital swelling, asthma and chronic rhinosinusitis. The patients' lung physiology was consistent with airway obstruction. Diagnosis was established by periorbital biopsy. All patients received oral corticosteroids for their periorbital swelling, without significant clinical response. Two patients received oral methotrexate, with nearly complete resolution of periorbital swelling. A third patient received oral azathioprine, without clinical response. The three patients had improvement of their asthma with inhaled steroids/long-acting bronchodilator, and immunosuppressive medication. A triad consisting of periorbital swelling, asthma and chronic rhinosinusitis should raise the suspicion of adult-onset asthma and periocular xanthogranuloma. Oral methotrexate should be considered as an alternative to corticosteroids in the treatment of this disorder.

Adult-Onset Asthma and Periocular Xanthogranulomas in a Patient With Lymphoplasmacytic Sclerosing Pancreatitis

The clinical syndrome of adult-onset asthma and periocular xanthogranulomas represents a rare systemic autoimmune disorder that has not been previously associated with autoimmune pancreatitis. Herein, we describe the case report of a 61-year-old man with the unique clinical association of adult-onset asthma, atopy, and periocular xanthogranulomas, who had previously been diagnosed with lymphoplasmacytic sclerosing pancreatitis. We have previously reported the case of a patient with lymphoplasmacytic sclerosing pancreatitis that developed postoperative inflammatory pseudotumor of the orbit. These unique, extrapancreatic, ocular manifestations of this disease add to the published observations that it is a systemic disorder with the potential for multiorgan involvement that may have a spectrum of clinical manifestations that includes adult-onset asthma and periocular xanthogranulomas.