A 57-year-old woman was diagnosed with SS-DE and pemphigus vulgaris at another hospital and had been experiencing severe subjective symptoms such as dryness, ocular pain, and foreign body sensation in both eyes. Although she was treated with sodium hyaluronate (SH) ophthalmic solution 0.3% (Hyalein  mini, Santen Pharmaceutical Co., Ltd., Osaka, Japan) and artificial tears, her symptoms did not improve at all. She performed frequent instillation of the SH ophthalmic solution 10-15 times daily because of severe symptoms.
In April 2014, she visited the Department of Ophthalmology, Nihon University Itabashi Hospital. The Dry Eye-related Quality of life Score (DEQS) was used to assess the subjective score of the patient. The DEQS at the baseline observational point and 5 weeks after initial treatment was 93.3 and 88.3, respectively. Slit-lamp examination of objective findings of ocular surface showed conjunctival swelling with follicular formation in the upper palpebral conjunctiva (fig. 1a) and SPK with fluorescein staining in the lower mid-peripheral cornea (fig. 1b). Blepharitis with skin erosion caused by pemphigus vulgaris was also observed (fig. 1c), and blinking imperfection might thereby occur. Abnormalities of tear dynamics were shown by Schirmer's test values of 3 mm in right eye and 5 mm in left eye, and tear brake-up time (TBUT) of 7 s in right eye and 8 s in left eye. The fluorescein staining was scored as follows. The ocular surface was divided into three areas (nasal conjunctiva, cornea, and temporal conjunctiva) that were assessed for fluorescein staining. Each area was judged a staining score from 0 (no damage) to 3 (severe damage) points, and the total score of fluorescein staining was calculated ranging from 0 to 9 points. Fluorescein scores of the right and left eyes were 1 and 1 point, respectively.
The patient was treated with a combination of rebamipide ophthalmic suspension four times daily and fluorometholone ophthalmic suspension (Flumetholon  ophthalmic suspension 0.1%, Santen Pharmaceutical Co., Ltd.) once daily for dry eye. As for the SH instillation, frequent instillation was changed to twice-daily instillation after the initial presentation. Blepharitis was treated with dexamethasone ophthalmic ointment (Santezon  ophthalmic ointment 0.05%, Santen Pharmaceutical Co., Ltd.). After having been treated with dexamethasone ophthalmic ointment for 1 week, the blepharitis treatment was continued using white petroleum gel (PROPETO ; Maruishi Pharmaceutical; Tokyo, Japan).
The Schirmer's test value, TBUT, and fluorescein score did not improve after 3 months with combined treatment for dry eye (fig. 1d). However, her subjective symptoms including ocular pain improved dramatically; DEQS score (93.3 to 88.3) and expression levels of ocular surface mucin also improved (shown in fig. 1e, f). MUC5AC at baseline point showed an extremely low level and was restored by the combined treatment to the levels comparable to those in healthy subjects. MUC16 also gradually increased by these treatments.