Letter to the Editor
In a recent issue of this journal, Kao et al (1) reported a patient with lupus erythematosus profundus (LEP) who presented with a violaceous periocular discoloration of the right upper and lower eyelids. Precontrast orbital T1 MRI showed diffuse loss of fat signal, and postcontrast fat-suppressed T1 MRI showed enhancement of the orbital fat and extraocular muscles. After treatment with oral prednisone, the patient developed dramatic orbital soft tissue lipoatrophy and enophthalmos.
We recently examined a patient who displayed another neuro-ophthalmic manifestation of LEP: central retinal artery occlusion (CRAO). MRI showed intraconal fat and intraorbital optic nerve sheath inflammation on the affected side, which may have been contributory.
A 51-year-old African American woman with history of biopsy-proven LEP for the past 20 years presented with acute loss of vision in the left eye. A punch biopsy of a left arm lesion 2 years earlier had shown hyperkeratosis, basement membrane thickening with basilar vasculopathy, perivascular inflammation in the dermis, sclerosing septal and hyalinizing lobular panniculitis with deep nodular lymphoid aggregates, and rare lymphoid follicles consistent with a diagnosis of LEP (Fig. 1). There was disfiguring hyperpigmentation and surface irregularity of her nose and fingertips with depressed lipoatrophic areas in the left midface and left arm.
Visual acuity was 20/20 in the right eye and counting fingers in the left eye, with a left relative afferent pupillary defect. Extraocular motility was full in both eyes, and the eyes appeared to be aligned. There was no proptosis or resistance to globe retropulsion. Anterior segment examination and intraocular pressures were normal bilaterally. Fundus examination was normal in the right eye but showed cloudy retinal swelling and a cherry red spot in the left macula consistent with CRAO. Neurological examination was otherwise normal.
Postcontrast fat-suppressed axial and coronal MRI showed abnormal enhancement of the preseptal and intraconal soft tissue and intraorbital optic nerve sheath and enlargement of all extraocular muscles in the left orbit (Fig. 2). Despite a course of methylprednisone, ophthalmologic examination and MRI findings remained unchanged 2 months later. She was slowly weaned off the corticosteroid.
We presume that the orbital inflammation in our patient led to the CRAO. Such a phenomenon has often been reported in other orbital inflammatory conditions, such as optic perineuritis (2) and idiopathic orbital inflammation (3). It has been reported only once in LEP in a patient who developed ischemic optic neuropathy and CRAO which led, months later, to melting of the entire orbital contents, including the eye (4)!
A unique aspect of our case is the inclusion of orbital inflammatory findings on MRI. This inflammatory response presumably led to CRAO and underscores the vision-threatening nature of LEP.
1. Kao TY, Yoon MK, McCulley TJ, Ruben BS, Hwang TN. Acquired enophthalmos in lupus erythematosus profundus. J Neuroophthalmol. 2010;30:64–66
2. Spierer O, Ben Sira L, Leibovitch I, Kesler A. MRI demonstrates restricted diffusion in distal optic nerve in atypical optic neuritis. J Neuroophthalmol. 2010;30:31–33
3. Foroozan R. Combined central retinal artery and vein occlusion from orbital inflammatory pseudotumour. Clin Experiment Ophthalmol. 2004;32:435–437
4. Arthurs BP, Khalil MK, Chagnon F, Lindley SK, Anderson DP, Burnier M Jr. Orbital infarction and melting in a patient with systemic lupus erythematosus. Ophthalmology. 1999;106:2387–2390