Periocular Xanthogranulomas, Reactive Polyclonal Lymphofollicular Hyperplasia of the Orbit and Allergic Bronchial Asthma
Kühnel B.1, Conway R. M.1, Kirchner T.2, Holbach L. M.1
1Dptm. of Ophthalmology and Eye Hospital, 2Department of Pathology, Univ. Erlangen-Nuremberg, Erlangen
Purpose: To highlight the clinical significance and differential diagnostic features of periocular xanthogranuloma through the presentation of a patient.
Method: Single interventional case report presenting clinical, radiologic and histopathologic findings.
Results: A 31-year-old female presented with slowly progressive bilateral lid swelling associated with yellow-coloured skin deposits of the eyelids first noted 4 years prior to presentation. These changes were accompanied by a background of chronic allergic bronchial asthma with mild eosinophilia. CT and ultrasound scans of the orbit showed diffuse infiltrative deposits involving the anterior orbits and lacrimal glands. Subsequent orbital and eyelid skin biopsies revealed a polyclonal lymphofollicular hyperplasia of the orbits and cutaneous xanthogranulomas respectively. The differntial diagnosis would include necrobiotic xanthogranuloma, Erdheim-Chester-disease and isolated xanthogranuloma. A course of systemic steroids resulted in complete resolution of the orbital desease.
Conclusions: A precise diagnostic evaluation of periocular xanthogranulomas is of practical significance as this entity may be associated with lifethreatening systemic illness.
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