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CORRESPONDENCE
Year : 2019  |  Volume : 64  |  Issue : 6  |  Page : 499-500
Anterior scleritis in a patient of pemphigus vulgaris while on immunosuppressive treatment


1 Department of Ophthalmology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India
2 Department of Dermatology, STD and Leprosy, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh, India

Date of Web Publication7-Nov-2019

Correspondence Address:
Brijesh Takkar
Department of Ophthalmology, All India Institute of Medical Sciences, Bhopal, Madhya Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijd.IJD_318_18

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How to cite this article:
Dube M, Takkar B, Asati DP, Jain S. Anterior scleritis in a patient of pemphigus vulgaris while on immunosuppressive treatment. Indian J Dermatol 2019;64:499-500

How to cite this URL:
Dube M, Takkar B, Asati DP, Jain S. Anterior scleritis in a patient of pemphigus vulgaris while on immunosuppressive treatment. Indian J Dermatol [serial online] 2019 [cited 2019 Nov 19];64:499-500. Available from: http://www.e-ijd.org/text.asp?2019/64/6/499/270544




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 2]a. 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

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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

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The whole lesion had dramatically resolved within 3 days with no recurrence of fever [Figure 2]b. 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.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Messent AJ, Blissett MJ, Smith GL, North AJ, Magee A, Foreman D, et al. Expression of a single pair of desmosomal glycoproteins renders the corneal epithelium unique amongst stratified epithelia. Invest Ophthalmol Vis Sci 2000;41:8-15.  Back to cited text no. 1
    
2.
Suami M, Kato M, Koide K, Usami Y, Hata N, Machida H, et al. Keratolysis in a patient with pemphigus vulgaris. Br J Ophthalmol 2001;85:1263-4.  Back to cited text no. 2
    
3.
Bystryn JC, Rudolph JL. Pemphigus. Lancet 2005;366:61-73.  Back to cited text no. 3
    
4.
Zeeli T, Bar-Ilan E, Habot-Wilner Z, Sprecher E. Anterior scleritis associated with pemphigus vulgaris. Ocul Immunol Inflamm 2019;27:497-8.  Back to cited text no. 4
    
5.
Mehta M, Dacey M, Stephen Foster C. Recurrent conjunctivitis and scleritis secondary to coexistent conjunctival pemiphigus vulgaris and cryptic herpes simplex infection: A case report. Ocul Immunol Inflamm 2010;18:454-6.  Back to cited text no. 5
    


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