Frosted branch angiitis, neuroretinitis as initial ocular manifestation in Behçet disease.

Behçet disease is an idiopathic, multisystem disorder characterized by recurrent episodes of orogenital ulceration and vasculitis of the veins and arteries of all calibers. Ocular involvement may affect the conjunctiva, sclera, uveal tract, vitreous, blood vessels, and retina. Many theories have pointed toward an autoimmune response behind its pathogenesis, which may be triggered by exposure to an infectious agent. Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. The disease may be idiopathic in a majority of the cases or may be associated with ocular and systemic pathology. Association between Behηet disease, Frosted branch angiitis, and neuroretinitis is not reported in literature. This uncommon combination reflects the varied systemic and ocular manifestations in Behçet disease, especially in patients who are not diagnosed and treated in time. We hereby report a case of bilateral frosted branch angiitis and neuroretinitis in a young male from Middle-east, suffering from Behçet disease.

Although uveitis and vasculitis are common features of Behçet disease, the frosted branch angiitis variant is an uncommon manifestation of this multisystem disorder. We have found only one item in Pubmed under the heading of ‘Frosted branch angiitis in Behçet disease’. To the best of our knowledge, the bilaterality of frosted branch angiitis and its sequel at an interval of two years has not been reported so far.

Case Report

A 28-year-old male patient referred with a history of an abrupt onset of floaters and shadows in the left eye, since five days. From records it was found that he had a similar episode in the right eye in, 2007, which was treated outside this hospital. Systemically, he has had recurrent mouth and genital ulcers, and skin lesions in the form of erythema nodosum.

His best corrected visual acuity on presentation was 20/100 and 20/20 in right and left eyes, respectively. The fundus image of right eye, in 2007, showed features of frosted branch angiitis and retinal hemorrhages [Fig. 1a]. Present examination of the right eye [Fig. 1b] revealed superior tractional retinal detachment and atrophic macular scar in the right eye, representing sequelae of the previous inflammation. The left eye showed papillitis, macular exudates, retinal edema with hemorrhages, and characteristic diffuse retinal perivascular sheathing, involving whole segments of both arterioles and venules in the left eye, suggestive of occlusive vasculitis, typical of frosted branch angiitis [Fig. 2a]. The anterior segment was normal. Fluorescein angiography (FA) showed delayed arteriovenous filling and widespread areas of capillary nonperfusion [Fig. 2b].

Figure 1

(a) Right eye (2007): Frosted branch angiitis with retinal hemorrhages. (b) Present fundus picture (2009) showing superior tractional retinal detachment and atrophic macular scar, representing sequelae of old inflammation

Figure 2

(a) Left eye (2009): Frosted branch angiitis with retinal hemorrhages. (b) Fluorescein Angiography showing multiple areas of nonperfusion. (c) Resolved inflammation following intravenous methylprednisone therapy and disease-modifying anti-rheumatic drugs

Investigations included biochemistry, serum proteins (C3, C4), coagulation, autoimmunity (anti-cardiolipin antibodies, anti-phospholipids antibodies, and DNA double stranded antibody), immunofluorescence (c- and p- anti-neutrophilic cytoplasmic antibodies), and sensory and motor nerve studies, which were within normal range. Magnetic resonance imaging (MRI) of the brain revealed few small bi-frontal subcortical white matter lesions suggestive of demyelinating vasculitis disease, correlating with Behçet disease.

On the basis of the history, clinical features, and FA findings, a diagnosis of frosted branch angiitis with neuroretinitis, secondary to Behçet disease, was made. An MRI further supported the diagnosis.

The patient was given a three-days course of intravenous methylprednisone (1 gm daily) and was put on disease modifying anti-rheumatic drugs, colchicine 0.5 mg daily and silymarin 75 mg twice daily. Two weeks later, his clinical picture showed a dramatic improvement in the inflammation of the disc, vessels, and reduction of macular exudates [Fig. 2c].

Discussion

Behçet disease is an idiopathic multisystem disorder, characterized by recurrent episodes of orogenital ulceration and vasculitis.[1] Frosted branch angiitis was originally described in the Japanese literature by Ito, in 1976, in a six-year-old child.[2] It is primarily a rare disease, manifested by severe vascular inflammation, sheathing, retinal edema, and retinal hemorrhages.[3]

Frosted branch angiitis can be associated with ocular and systemic diseases.[4] Cytomegalovirus retinitis, Acquired immunodeficiency syndrome, retinitis, toxoplasmic chorioretinitis, and central retinal venous occlusion are frequent ocular associations, while systemic lupus erythematosus, Crohn's disease, large cell lymphoma, and acute lymphoblastic leukemia have been described as systemic associations.[2] Frosted branch angiitis in association with Behçet disease is rare and has been reported in a pregnant female.[5] To the best of our knowledge, frosted branch angiitis with neuroretinitis has not been reported in a young male patient in both eyes, two years apart.