Although conjunctival sarcoidosis mostly presents as multiple nodules resembling follicular conjunctivitis, other conjunctival manifestations including conjunctival deposit, large conjunctival tumor and multilobular limbal nodules have been reported [
1‐
3,
5‐
7]. The differential diagnosis of limbal hypertrophy includes vernal keratoconjunctivitis, atopic keratoconjunctivitis, allergic conjunctivitis, superior limbic keratoconjunctivitis, trachoma, ligneous conjunctivitis, soft contact lens complication, and conjunctival tumors such as papilloma, lymphoma and intraepithelial neoplasia [
8,
9]. However, sarcoidosis has rarely been considered in the differential diagnosis, and in fact, our patient was referred to us with an impression of conjunctival neoplasia. In most cases of conjunctival sarcoidosis, the granulomatous inflammation results in conjunctival nodules. However, in our patient, there was a large amount of granulomatous infiltration around the superior perilimbal area, and it may have led to the limbal conjunctival hypertrophy.
Cyclosporine is known to be useful in various ocular surface inflammatory diseases, such as ocular rosacea, dry eye disease, atopic keratoconjunctivitis, graft-versus-host disease and herpetic stromal keratitis, due to its anti-inflammatory and immunomodulatory action [
10]. Although the pathogenesis of sarcoidosis is still unclear, cluster of differentiation (CD) 4 T cells appear to play an important role in granuloma formation [
1,
2]. Accordingly, cyclosporine is expected to be useful for its selective inhibitory effect on CD4+ T lymphocyte proliferation via inhibition of interleukin-2 receptor expression [
1,
3]. Oral cyclosporine is shown to be effective in vision-threatening ocular sarcoidosis as well as refractory systemic sarcoidosis [
3]. Topical cyclosporine was also effective in the treatment of conjunctival sarcoidosis in steroid-responders [
2,
3]. We also chose topical cyclosporine instead of steroids due to pre-existing glaucoma in our patient. Unlike previous cases [
2,
3], our patient’s serum ACE level was elevated, and normalized in response to the cyclosporine treatment. In patients with sarcoidosis, serum ACE levels are elevated due to marked synthesis by epithelioid cells, thus the ACE level reflects the mass of ACE-producing granuloma cells [
11]. Therefore, although an elevated serum ACE level is not a specific finding for sarcoidosis, serial measurements of serum ACE level are useful in the evaluation of treatment response. In our patient, her elevated ACE level normalized with the disappearance of conjunctival lesions, which suggests suppression of disease activity with the use of topical cyclosporine.