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Show Acquired Enophthalmos in Lupus Erythematosus Profundus Tina Y. Kao, BS, Michael K. Yoon, MD, Timothy J. McCulley, MD, Beth S. Ruben, MD, Thomas N. Hwang, MD, PhD Abstract: Lupus erythematosus profundus (LEP) is an uncommon subtype of lupus erythematosus. A 76-year old man presented with inflammation of the eyelids and underlying orbital soft tissue. A biopsy disclosed in-flammation and atrophy of the orbital fat consistent with LEP. Systemic corticosteroid treatment produced resolu-tion of the inflammation. but as the edema subsided, enophthalmos became apparent. LEP should be consid-ered in patients with a characteristic rash and orbital inflammation and may cause acquired enophthalmos. Journal of Neuro-Ophthalmology 2010;30:64-66 doi: 10.1097/WNO.0b013e3181c25698 2010 by North American Neuro-Ophthalmology Society A76-year-old Hispanic man presented with a 3-month history of a progressive right periocular discoloration. A violaceous lesion appeared on the right lower eyelid, together with a dull periocular ache, and gradually extended to involve the ipsilateral cheek and upper eyelid. The pain lasted only an hour. He had had Hashimoto thyroiditis, diverticulitis, and chronic hepatitis C. We noted diffuse red-violet discoloration of the right upper and lower eyelids with palpable induration (Fig. 1). The inferior portion of the upper eyelid was spared. He had trace conjunctival injection in the ipsilateral eye. The rest of the ophthalmologic examination was normal. FIG. 1. A. Dark discoloration of the right upper and lower eyelids in a patient with lupus erythematosus profundus, a variant of systemic erythematosus. The inferior portion of the upper eyelid, being relatively protected from sun exposure, is relatively less discolored. B. Precontrast T1 coronal MRI without fat signal suppression shows partial loss of fat signal within the orbit. C. Postcontrast T1 coronal MRI with fat signal suppression shows enhancement of the orbital fat and extraocular muscles. D. After treatment with systemically administered corticosteroid, there is right enophthalmos. Departments of Ophthalmology (TYK, KYM, TJM) and Dermatology and Pathology (BSR), University of California San Francisco, San Francisco California; Department of Ophthalmology (TNH), Kaiser Permanente, Redwood City, California. Address correspondence to Thomas N. Hwang, MD, Department of Ophthalmology, Kaiser Permanente, 1150 Veterans Blvd, Redwood City, CA 94063; E-mail: tnhwang@gmail.com 64 Kao et al: J Neuro-Ophthalmol 2010; 30: 64-66 Photo Essay Hematocrit was 37.5%, the erythrocyte sedimentation rate was 85 mm/h, the antinuclear antibody (ANA) titer was 1:320 with a diffuse pattern, double-stranded (ds) DNA antibody was 44 IU/ml, and serum gamma globulin was 2.3 g/dL. Leukocyte count and differential, myeloperoxidase antibody and anti-proteinase antibody, angiotensin-converting enzyme, cryoglobulin, and extractable nuclear antigens SSA, SSB, Sm, and ribonucleoprotein (RNP) were normal. T1 orbital MRI demonstrated a diffuse loss of fat signal and enhancement of the orbital fat and extraocular muscles (Fig. 1). Biopsy of the lower and upper eyelid skin, orbicularis oculi, and lateral rectus muscles, orbital septum, and extraconal and intraconal orbital fat disclosed a predominantly perivascular lymphoplasmacytic infiltrate. Cutaneous sections showed fat hyalinization of the subcutis and an atrophic vacuolar interface reaction as well as hyperkeratosis with colloid bodies. Melanophages were prominent in the dermis. These histopathologic findings were considered consistent with lupus erythematosus profundus (LEP) (Fig. 2). Treatment with 60 mg oral prednisone daily was started, and skin lesions resolved within 2 weeks. Diffuse periocular lipoatrophy resulting in acquired enophthalmos of 3 mm with depression of the superior sulcus was observed. The enophthalmos stabilized by the third treatment week (Fig. 1). One month after initiation of treatment, during the corticosteroid taper, the patient developed acute diver-ticulitis and subsequently died of related complications. There are several variations of lupus erythematosus. A relatively common variety is discoid lupus erythematosus (DLE), characterized by cutaneous plaque-like inflammation. In 1883, Kaposi (1) described a variant of DLE now known as LEP. It is characterized by extension to the subcutaneous fat and panniculitis with or without overlying DLE-associated skin changes. LEP can also precede, follow, or occur independently of discoid or systemic forms of lupus erythematosus. Although the disease is considered benign, it can cause significant morbidity, including disfigurement from lipoatrophy after resolution of the lesions. LEP lesions typically involve the scalp, cheeks, arms, shoulders, breast, buttocks, or thighs but rarely affect periorbital tissues (2). Diagnosis of LEP is based on clinical and histologic findings. However, subcutaneous involvement, confirmed with biopsy, is necessary to establish the diagnosis. Although results are often normal, serologic analysis may show a positive ANA titer. Anti-dsDNA antibodies may be elevated (2). LEP is often associated with other autoimmune disorders such as Hashimoto's thyroiditis, as seen in our patient. Although there have been several reports of DLE lesions affecting the eyelids, periocular involvement in LEP is uncommon, occurring in only 2%-5% of patients (3). Patients may have periocular edema (4-7) or proptosis (5,8). There is one report of severe orbital involvement, which progressed to complete orbital infarction and melting (9). The unusual orbital imaging findings include loss of the fat signal on T1 MRI, consistent with the histologic finding of loss of orbital fat, together with extensive inflammatory and fibrous infiltrate within the septa between fat lobules (Fig. 2). In our patient, this inflammation was apparent as intense enhancement on fat-suppressed postcontrast T1 MRI. During the period of inflammatory swelling, there was no enophthalmos. As the inflammatory infiltrate FIG. 2. A. Histopathology of eyelid skin biopsy demonstrates atrophic epidermis, subtle vacuolar interface changes (arrowhead) with colloid bodies, and an infiltrate of lymphocytes in the dermis, where melanophages (arrow) are also conspicuous. B. Intraconal orbital fat biopsy demonstrates lymphoplasmacytic infiltration (arrow) and "hyalinization" of fibrous septae between adipocytes (arrowhead). C. Biopsy of lateral rectus muscle biopsy demonstrates perivascular lymphoplasmacytic infiltrate (arrow) (hematoxylin and eosin stain). Photo Essay Kao et al: J Neuro-Ophthalmol 2010; 30: 64-66 65 regressed with corticosteroid treatment, enophthalmos developed, most likely unveiling the preexisting fat atrophy that had been masked by orbital edema. We cannot exclude the possibility that systemic corticosteroid therapy contrib-uted to fat atrophy, but no local corticosteroid therapy had been administered, and no fat atrophy was ever observed in the contralateral orbit or elsewhere. Despite pathologic abnormalities in the extraocular muscles on imaging and biopsy, the patient had normal extraocular motility during all stages of disease and treatment. Moreover, despite intense orbital inflammation, our patient reported no persistent pain. REFERENCES 1. Kaposi M. Pathologie und Therapie der Hautkrankheiten in Vorlesungen fu¨r praktische Aerzte und Studirende. 2nd ed. Baltimore: Urban & Schwarzenberg; 1883: 642. As cited in Sanchez NP, Peters MS, Winkelmann RK. The histopathology of lupus erythematosus panniculitis. J Am Acad Dermatol 1981;5;673-80. 2. Fraga J, Garcı´a-Dı´ez A. Lupus erythematosus panniculitis. Dermatol Clin 2008;26:453-63. 3. Kundig TM, Tru¨eb RM, Krasovec M. Lupus profundus/ panniculitis. Dermatology 1997;195:99-101. 4. Sheehan-Dare RA, Cunliffe WJ. Severe periorbital oedema in association with lupus erythematosus profundus. Clin Exp Dermatol 1988;13:406-17. 5. Nowinski T, Bernardino V, Naidoff M, et al. Ocular involvement in lupus erythematosus profundus (panniculitis). Ophthalmology 1982;89:1149-54. 6. Jacyk WK, Bhana KN. Lupus erythematosus profundus in black South Africans. Int J Dermatol 2006;45:717-21. 7. Lodi A, Pozzi M, Agostoni A, et al. Unusual onset of lupus erythematosus profundus. Br J Dermatol 1993;129: 96-107. 8. Magee KL, Hymes SR, Rapini RP, et al. Lupus erythematosus profundus with periorbital swelling and proptosis. J Am Acad Dermatol 1991;24:288-90. 9. Arthurs BP, Khalil MK, Chagnon F, et al. Orbital infarction and melting in a patient with systemic lupus erythematosus. Ophthalmology 1999;106:2387-90. Photo Essay 66 Kao et al: J Neuro-Ophthalmol 2010; 30: 64-66 |
