Siebert S, Srinivasan U. Proptosis can be the presenting feature of systemic lupus erythematosus. Ann Rheum Dis. 2004 Aug;63(8):908-9.
“Patients with SLE can present with a variety of ocular manifestations, with keratoconjunctivitis sicca being the most common ocular finding. Retinal involvement, usually due to vascular damage, is reported to occur in 10% of patients with SLE and is an important manifestation as it may reflect systemic disease activity. In addition to our case, several other reports of SLE presenting with proptosis due to orbital pseudotumour have been published. Orbital pseudotumour, part of the spectrum of idiopathic orbital inflammation, is a non-granulomatous inflammatory disorder that may affect any structure in the orbit. It is defined according to the orbital structures affected, with the presenting symptoms reflecting the degree of inflammation and the location of the inflamed tissue. Patients with an acute presentation may complain of generalised malaise, in addition to ocular symptoms, but are generally afebrile, unlike our patient. Infections such as orbital cellulitis can mimic the features of orbital pseudotumour and should be excluded in patients with pyrexia before any immunosuppressive treatment is started. The acute presentation of orbital inflammation is usually with an abruptly painful eye, commonly associated with impaired vision. Mass effect in the orbit tends to be more pronounced in chronic cases, while myositis is suggested by painful diplopia exacerbated by eye movements. The diagnosis can usually be made on clinical and radiological (MRI or CT) grounds, avoiding the need for biopsy in most cases.”
Gómez-Puerta JA, Levy S, Khamashta MA, Hughes GR. Periorbital oedema in systemic lupus erythematosus. Lupus. 2003;12(11):866-9.
“Periorbital oedema is an unusual cutaneous manifestation reported in only a few cases in patients with systemic lupus erythematosus (SLE). Eyelids are affected in lupus patients in different ways, including infections, subcutaneous nodules, angioedema, secondary dermatomyositis, periorbital mucinosis and edema due to hypoalbuminemia related to nephrotic syndrome or hepatic disease. We describe here three women with SLE and periorbital oedema in the absence of any identifiable cause.”
Maeguchi M, Nogita T, Ishiguro N, Nihei H, Kawashima M. Periorbital oedema and erythema in systemic lupus erythematosus. Br J Dermatol. 1996 Mar;134(3):601-2.
“Facial involvement, in the form of a butterfly rash and discoid lesions, in systemic lupus erythematosus (SLE) are common, but periorhital oedema and erythema as the sole manifestation of SLE are rare. We describe an unusual case of SLE presenting as bilateral eyelid oedema and erythema
associated with lupus nephritis. The histological findings of the eyelid showed the presence of marked dermal mucin. The administration of prednisone reduced the oedema and erythema without scarring or pigmentation.”
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Created 2/27/14, reviewed & updated 3/12/14.