Necrotizing Granulomatous Dacryoadenitis With Non-Necrotizing Granulomatous Scar Hypertrophy: Two Histological Variants of Sarcoidosis in the Same Patient

Abstract

Orbital involvement with histologic necrosis is a rare manifestation of systemic sarcoidosis. The authors present a case of necrotizing dacryoadenitis in addition to non-necrotizing granulomas in a hypertrophic scar that is consistent with a diagnosis of sarcoidosis. A 60-year-old female presented with 2 months of painless right upper eyelid fullness and ptosis. CT imaging demonstrated right greater than left lacrimal gland enlargement. A biopsy demonstrated necrotizing granulomatous inflammation of the lacrimal gland. Additional workup was negative for infectious or lymphoproliferative disease. On further investigation, the patient noted thickening of a longstanding abdominal scar, and a subsequent punch biopsy of the scar demonstrated non-necrotizing granulomas suggestive of scar sarcoidosis. CT chest identified mediastinal lymphadenopathy. A diagnosis of sarcoidosis was determined. The authors thereby present an unusual case of 2 histologic variants of sarcoidosis presenting with necrotizing granulomatous dacryoadenitis and non-necrotizing scar granulomas.

A Case of Orbital Sarcoidosis with Caseating Granulomatous Inflammation

“…6 One past case similarly reported a patient with 2 histologic variants of sarcoidosis, caseating granulomatous inflammation of the lacrimal gland and noncaseating granulomas of a longstanding abdominal scar, along with mediastinal lymphadenopathy, although a mediastinal lymph node biopsy was not obtained. 17 These 2 presentations may suggest that contrary to past algorithms which suggest ruling out sarcoidosis purely based on the initial presenting mass biopsy findings (pulmonary or nonpulmonary), 9,18 screening for additional organ involvement and biopsy of involved lymph nodes may be considered before the final diagnosis. It also further supports the importance of systemic investigation including chest radiography, negative tuberculin test or interferon-gamma releasing assay, serum angiotensin-converting enzyme, and elevated serum lysozyme, even when the biopsy is not entirely characteristic of sarcoidosis.…”