Lupus erythematosus profundus masquerading as idiopathic orbital inflammatory syndrome

Abstract

Idiopathic orbital inflammatory syndrome (IOIS) is a nonspecific inflammation of orbital tissue. As it is a diagnosis of exclusion, systemic testing and, at times biopsy, is utilized to rule out other inflammatory etiologies. Since some inflammatory etiologies that masquerade as typical IOIS can be vision or life threatening, it is important to consider these diagnoses. Systemic lupus erythematosus (SLE) is a chronic systemic autoimmune process that can affect the eye and visual system in 20% of individuals. In this idiopathic process, the deposition of pathogenic autoantibodies and immune complexes damage tissues and cells. Some common ocular manifestations of SLE include keratoconjunctivitis sicca, periocular skin lesions, orbital inflammation, retinal hemorrhages and vasculitis, retinal vaso-occlusive disease, iritis, scleritis, optic neuritis and optic neuropathy. One rare clinical entity in the SLE spectrum is panniculitis, also known as lupus erythematosus profundus (LEP), which is a nodular inflammation of adipose tissue. Panniculitis involving orbital structures as the primary presenting symptom of SLE is quite unusual and has only rarely been previously reported in the literature and has not been reported presenting as IOIS. This uncommon presentation can make the diagnosis more difficult. We describe a patient who had presented with ptosis evolving to orbital inflammation, which was consistent with IOIS by laboratory and histologic examinations. The patient later developed extensive panniculitis and a final diagnosis of LEP was made.