Anterior Scleritis in a Patient of Pemphigus Vulgaris while on Immunosuppressive Treatment

Sir,

A 56-year-old male presented with a painful and red left eye for 4 days, which was preceded by fever. The patient was a known case of pemphigus vulgaris (PV) [Figure 1] and was being treated with oral prednisolone, azathioprine, and rituximab (first dose 10 days prior). Visual acuity was 6/6 bilaterally. A mildly raised ill-defined lesion was seen temporal to the limbus with intense congestion of the deep scleral vessels [Figure 2a]. Overlying conjunctiva appeared necrotic with minimal nonpurulent discharge. The patient was suspected to have infective scleritis, and a swab of the inferior conjunctival fornix was sent for bacterial culture and drug sensitivity. Frequent topical moxifloxacin (0.5%) and an oral combination of amoxicillin (500 mg) and clavulanic acid (125 mg) were initiated.

Figure 1.

Dry, crusted erosions of pemphigus vulgaris can be seen on anterior chest wall in healing phase

Figure 2.

(a) Anterior scleritis is noticeable in lateral aspect of the left eye. (b) Complete resolution of lesion is seen 3 days after initiating treatment

The whole lesion had dramatically resolved within 3 days with no recurrence of fever [Figure 2b]. Bacterial culture grew coagulase-negative Staphylococcus. The topical antibiotic was continued till 15 days, while oral therapy was stopped after 7 days. The patient remained stable till a follow-up of 1 month.

PV is an autoimmune disease, in which antibodies are formed against adhesion molecules desmoglein 1 or desmoglein 3, resulting in blistering of the skin and mucous membrane. The condition is characterized by a loss of adhesion between epithelial cells, resulting in suprabasal acantholysis and the presence of epithelial and serum autoantibodies. Ocular involvement in PV may be explained by the presence of desmoglein 3 in ocular epithelium.[1] The most common feature is noncicatricial conjunctivitis, and in rare cases, erosions on the eyelids and the conjunctiva may also be observed. However, some cases of “atypical” presentation characterized by conjunctival scarring and corneal perforation have also been reported.[2] Ocular involvement has been reported as a signal of severe disease and tends to occur several months after the onset of skin or other mucosal lesions. However, there is evidence to suggest that ocular symptoms can precede the onset of skin and mucus membrane lesions, suggesting that ocular involvement may be underdiagnosed.[3]

The presence of scleritis in a patient of PV is very rare, and literature is limited to only a handful of cases. Zeeli et al.[4] presented a young PV patient with a recalcitrant disease who developed anterior nodular scleritis in 2017. The authors concluded that this severe condition should be considered in the differential diagnosis of any pemphigus patient who develops a painful red eye. In 2010, Mehta et al.[5] reported herpetic keratitis in a patient with cutaneous pemphigus in which herpetic scleritis occurred while the patient was on intermittent treatment with acyclovir for genital herpes. Coagulase-negative Staphylococcus are known to be present as conjunctival flora. Therefore, it is possible that a previously present conjunctival erosion in our patient's left eye paved the way for the microbes to breach conjunctival barrier of defense and reach the deeper sclera, while the prior long-term immune suppression (inclusive of steroids) allowed proliferation of the bacteria leading to scleritis. Due to the tendency of these patients to get infected, especially while on heavy immune suppression, aggressive management of “redness of the eye” is recommended.

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