Adult Onset Asthma and Periocular Xanthogranuloma: A Difficult Diagnosis

Daniela Reyes-Capό, Kimberly D. Tran, Juan Ayala-Haedo, Oliver Fischer, Sander A. Dubovy and Sara Wester

Department of Ophthalmology, University of Miami Miller School of Medicine, USA

^*Correspondance to: Sara Wester 

 PDF  Full Text DOI: 10.25107/2474-1647.1168

Abstract

Purpose: This case report describes the presentation, diagnosis, and treatment of adult onset asthma and periocular xanthogranuloma (AAPOX) in a 41 year-old male and highlights the diagnostic and therapeutic challenges of this disease. Observations: The authors report a case of a 41 year-old male with a 12 year history of episodic bilateral upper eyelid edema, and mechanical ptosis of uncertain etiology. He had previously undergone biopsy and failed trials of surgical excision, systemic corticosteroids, methotrexate, and radiation therapy. He was initially diagnosed in 2004 with bilateral idiopathic orbital inflammation by biopsy. Due to concomitant sinus congestion, shortness of breath, and hilar adenopathy on chest x-ray, the patient underwent endoscopic sinus surgery and transbronchial biopsy, which showed vague granulomatous chronic inflammation suggestive of sarcoidosis. Serologic workup was notable for peripheral blood eosinophilia (17%); however, infectious and rheumatologic serologies, including angiotensin converting enzyme (ACE) and antinuclear antibody (ANA), were unremarkable. The patient presented to the Bascom Palmer Eye Institute (BPEI) for evaluation due to recurrence of bilateral lid swelling. He underwent repeat biopsy which disclosed fibro vascular and adipose tissue containing xanthomatous infiltrate of foamy histiocytes without necrosis consistent with xanthogranuloma. The overall presentation was suggestive of AAPOX. The patient was started on methotrexate and long-acting bronchodilators, and at 8-month follow-up the patient reports improved left-sided peripheral vision, and continues to be followed by the Pulmonology, Rheumatology, and Oculoplastics services.Conclusion and Importance: AAPOX is a rare, non-Langerhans histiocytic disorder with characteristic orbital and systemic findings. This patient was followed for 12 years by multiple providers with innumerable work-up, biopsies, and imaging without a definitive diagnosis. This case illustrates the diagnostic challenges and important histopathologic clues in the diagnosis of this rare disease.

Keywords

Orbital mass; Eyelid swelling; Asthma; Xanthogranulomatous disease

Cite the article

Reyes-Cap? D, Tran KD, Ayala-Haedo J, Fischer O, Dubovy SA, Wester S. Adult Onset Asthma and Periocular Xanthogranuloma: A Difficult Diagnosis. Clin Surg. 2016; 1: 1168.