Título / Title

BEHÇET DISEASE PRESENTING AS A FROSTED ANGIITIS

Introdução / Purpose

To report an unusual case of frosted branch angiitis in Behçet disease.

Material e Método / Methods

Case report

Resultados / Results

A 21-years-old male presents with severe ocular pain and acute vision loss in both eyes, most pronounced on his left eye (LE). The patient reported non-specific immunodeficiency diagnosis 3 years ago, presented with oral and genitals ulcers, and cutaneous lesions, similar to erythema nodosum. His best corrected visual acuity (BCVA) on presentation was 20/200 on the right eye (RE) and LP on LE. Anterior segment biomicroscopy showed anterior chamber inflammation on RE, and neovascular glaucoma on LE. The fundus examination revealed in the RE, hyperemia and optic disc edema, macular edema, intense vascular tortuosity and vasculitis on the inferior branch, besides presence of multiple intraretinal hemorrhages characterizing frosted branch angiitis (Fig. 1, 2). Fundus examination on LE was impossible due means opacity. The diagnosis was frosted branch angiitis secondary to Behçet disease. The patient underwent to a laboratory and imaging investigation to exclude other diseases, including infectious etiologies. All came out within the normal ranges. Treatment was started with pulse therapy with intravenous methylprednisolone followed by oral corticoid (60mg/day). After 45 days BCVA improvement to 20/80 on RE and remained LP on OS. Fundus findings improved as well (Fig. 3). Complete healing of cutaneous lesions (Fig. 4) and oral ulcers were obtained.

Discussão e Conclusões / Conclusion

Frosted branch angiitis is characterized by vascular inflammation, sheathing, retinal edema, and retinal hemorrhages. Cytomegalovirus retinitis, acquired immunodeficiency syndrome retinitis, toxoplasmic retinitis are the most frequent ocular associations, while systemic lupus erythematosus has been described as systemic disorder associated. Although vasculitis is common in Behçet disease, the frosted branch angiitis variant is an uncommon manifestation of this disorder.

Palavras Chave

Angiitis; Behçet disease