Unilateral Eyelid Edema as Initial Sign of Orbital Sarcoidosis

Abstract

Introduction. Sarcoidosis is a rare multisystemic granulomatous inflammatory disease of unknown etiology affecting the respiratory system, skin, and eyes. Sarcoidosis outside the lacrimal gland is rare. The case study concerns a patient with a final diagnosis of orbital sarcoidosis. Case Report. A 37-year-old male patient went to the ophthalmic emergency room complaining of pain in the left eye, diplopia, and decreased visual acuity. An external eye examination showed hard and cold edema of the lower eyelid, ocular motility with limitation of adduction, and discreet ipsilateral proptosis. Magnetic resonance of the orbit showed left eye proptosis and thickening and increase of soft tissues associated with heterogeneous impregnation of contrast in the infralateral region of the left eyelid. A biopsy of the lesion showed a chronic inflammatory process, with numerous compact nonnecrotizing granulomas surrounded by lamellar hyaline collagen, providing histological confirmation of sarcoidosis. Discussion. A biopsy of the orbital tumor is essential for the diagnosis of sarcoidosis, in addition to the search for systemic findings such as hilar adenopathy or parenchymal lung disease found in 90% of patients.

Seltene Erkrankungen der Orbita

“…The term ‘sarcoid’ was introduced by Caesar W. Boeck because these lesions are histologically similar to sarcomas without being malignant. Effects in the lung with bihilary lymphadenopathy as well as skin and eyelid lesions are typical findings 155 156 157 .…”

“…orbital involvement possible as first manifestation: mostly lacrimal gland (often bilateral involvement); furthermore, orbital fatty tissue, optic nerve sheath (as part of neurosarcoidosis), efferent lacrimal ducts, or eye muscles 155 156 159 160 161 …”

“…depending on the affected structure, palpable tumor mass, eyelid swelling, ptosis, diplopia, exophthalmos, epiphora, sicca symptoms, or painful ophthalmoplegia 156 161 …”

“…in refractory cases or recurrence: immunosuppressive combination therapy, e. g., with methotrexate or azathioprine, or with biologicals such as infliximab or adalimumab 156 161 …”

“…Der Begriff „sarkoid“ wurde durch Caeser W. Boeck eingeführt, da die Läsionen histologisch Sarkomen ähneln, jedoch benigne sind. Klassisch sind ein Befall der Lungen und eine bihiläre Lymphadenopathie sowie Haut- und Augenlidläsionen 155 156 157 .…”