Periocular xanthogranuloma: A forgotten entity?

Charalampos Papagoras1, George Kitsos2, Paraskevi V Voulgari1, Anastasia K Zikou3, Maria I Argyropoulou3, Aikaterini Zioga4, Alexandros A Drosos11Rheumatology Clinic, Department of Internal Medicine; 2Department of Ophthalmology; 3Department of Clinical Imaging and Radiology, 4Department of Patholog...

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Autores principales: Charalampos Papagoras, George Kitsos, Paraskevi V Voulgari, et al
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Publicado: Dove Medical Press 2010
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spelling oai:doaj.org-article:148bc55969bd438f95fd97a3afed667f2021-12-02T05:56:21ZPeriocular xanthogranuloma: A forgotten entity?1177-54671177-5483https://doaj.org/article/148bc55969bd438f95fd97a3afed667f2010-03-01T00:00:00Zhttp://www.dovepress.com/periocular-xanthogranuloma-a-forgotten-entity-a4033https://doaj.org/toc/1177-5467https://doaj.org/toc/1177-5483Charalampos Papagoras1, George Kitsos2, Paraskevi V Voulgari1, Anastasia K Zikou3, Maria I Argyropoulou3, Aikaterini Zioga4, Alexandros A Drosos11Rheumatology Clinic, Department of Internal Medicine; 2Department of Ophthalmology; 3Department of Clinical Imaging and Radiology, 4Department of Pathology, Medical School, University of Ioannina, Ioannina, GreeceAbstract: 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.Keywords: periocular xanthogranuloma, adult-onset asthma, non-Langerhans histiocytoses, cyclophosphamide, methylprednisolone Charalampos PapagorasGeorge KitsosParaskevi V Voulgariet alDove Medical PressarticleOphthalmologyRE1-994ENClinical Ophthalmology, Vol 2010, Iss default, Pp 105-110 (2010)
institution DOAJ
collection DOAJ
language EN
topic Ophthalmology
RE1-994
spellingShingle Ophthalmology
RE1-994
Charalampos Papagoras
George Kitsos
Paraskevi V Voulgari
et al
Periocular xanthogranuloma: A forgotten entity?
description Charalampos Papagoras1, George Kitsos2, Paraskevi V Voulgari1, Anastasia K Zikou3, Maria I Argyropoulou3, Aikaterini Zioga4, Alexandros A Drosos11Rheumatology Clinic, Department of Internal Medicine; 2Department of Ophthalmology; 3Department of Clinical Imaging and Radiology, 4Department of Pathology, Medical School, University of Ioannina, Ioannina, GreeceAbstract: 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.Keywords: periocular xanthogranuloma, adult-onset asthma, non-Langerhans histiocytoses, cyclophosphamide, methylprednisolone
format article
author Charalampos Papagoras
George Kitsos
Paraskevi V Voulgari
et al
author_facet Charalampos Papagoras
George Kitsos
Paraskevi V Voulgari
et al
author_sort Charalampos Papagoras
title Periocular xanthogranuloma: A forgotten entity?
title_short Periocular xanthogranuloma: A forgotten entity?
title_full Periocular xanthogranuloma: A forgotten entity?
title_fullStr Periocular xanthogranuloma: A forgotten entity?
title_full_unstemmed Periocular xanthogranuloma: A forgotten entity?
title_sort periocular xanthogranuloma: a forgotten entity?
publisher Dove Medical Press
publishDate 2010
url https://doaj.org/article/148bc55969bd438f95fd97a3afed667f
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AT georgekitsos periocularxanthogranulomaaforgottenentity
AT paraskevivvoulgari periocularxanthogranulomaaforgottenentity
AT etal periocularxanthogranulomaaforgottenentity
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