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Middle East African Journal of Ophthalmology Middle East African Journal of Ophthalmology
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  Table of Contents 
Year : 2021  |  Volume : 28  |  Issue : 1  |  Page : 63-64  

Pemphigus vulgaris-associated anterior scleritis: A case study

1 Department of Dermatology, King Saud University, College of Medicine; Department of Dermatology, King Khalid University Hospital, Riyadh, Saudi Arabia
2 Department of Dermatology, King Saud University, College of Medicine, Riyadh, Saudi Arabia
3 Department of Surgery, Division of Ophthalmology, King Saud Bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia
4 Vitreoretinal Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia

Date of Submission04-Aug-2020
Date of Acceptance28-Jan-2021
Date of Web Publication30-Apr-2021

Correspondence Address:
Dr. Salim Alkeraye
King Saud University, College of Medicine, P.O. Box 11472 Riyadh 7805
Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/meajo.MEAJO_442_20

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Ocular involvement in pemphigus vulgaris (PV) is relatively rare. The conjunctiva and eyelids are considered the most common affected sites in ocular pemphigus. Scleritis is rarely reported as a manifestation of PV. We present a case report of anterior scleritis as a manifestation of PV and its response to rituximab therapy.

Keywords: Anterior scleritis, ocular pemphigus, pemphigus vulgaris

How to cite this article:
Alkeraye S, Alsalman H, Alhamzah A, Alsulaiman SM. Pemphigus vulgaris-associated anterior scleritis: A case study. Middle East Afr J Ophthalmol 2021;28:63-4

How to cite this URL:
Alkeraye S, Alsalman H, Alhamzah A, Alsulaiman SM. Pemphigus vulgaris-associated anterior scleritis: A case study. Middle East Afr J Ophthalmol [serial online] 2021 [cited 2022 May 18];28:63-4. Available from: http://www.meajo.org/text.asp?2021/28/1/63/315321

   Introduction Top

Pemphigus vulgaris (PV) is a rare, severe immune-mediated mucocutaneous disease. It is characterized by autoantibody production against intercellular adhesion molecules which results in loss of keratinocytes adhesions, mainly circulating IgG antibodies against desmoglein 3 or both desmoglein 1 and desmoglein 3.[1],[2] These molecules are members of the cadherins family, a cell adhesion molecule that binds epithelial cells to each other. Clinically, the loss of keratinocyte to keratinocyte adhesion will lead to painful erosions and blisters of the skin and mucous membranes including oral, nasopharyngeal, conjunctival, anal, and genital mucosae.[3],[4] Akhyani et al. reported an estimated ocular involvement of PV to be around 16.5% with conjunctivitis being the most common manifestation followed by erosion of the palpebral conjunctiva.[5]

   Case Report Top

A 45-year-old female, who is a known to have PV for the last 5 years, presented to the dermatology clinic with a 8-day history of redness of her right eye. The patient has been following for a recalcitrant PV, confirmed by histopathological studies and direct immunofluorescence, involving her oral, pharyngeal, and laryngeal mucosae, for which she received multiple therapies with varying success including systemic corticotherapy, azathioprine, and mycophenolate mofetil. One week before this presentation, the patient received the first dose of 1 g of intravenous rituximab due to persistent disease despite immunosuppression. Dermatological examination revealed multiple erosions on the buccal mucosa, soft palate, and upper and lower gingivae. Ophthalmic examination revealed visual acuity of 20/20 in both eyes with normal intraocular pressures. The left eye examination was unremarkable. The right eye showed severe diffuse redness with dilation of deep episcleral vessels associated with tenderness to palpation [Figure 1]. There was no discharge, follicles, or papillae on the palpebral conjunctiva. The cornea was clear with a quiet anterior chamber. The posterior segment was normal. The patient was thought to have a diffuse anterior nonnecrotizing scleritis secondary to PV and topical prednisolone acetate 1% drops were given. Besides, she received the second dose of rituximab. Two weeks later, her oral and ocular lesions completely regressed with no recurrence until last follow-up 2 years after treatment.
Figure 1: The right eye showed severe diffuse redness with dilation of deep episcleral vesselsepiscleral vessels

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   Discussion Top

Mucosal membranes are affected in the vast majority of patients with PV, with the oral cavity being the most common site affected and may represent the initial site of the disease.[6]

Ocular involvement in PV is considered rare.[1] However, there is no sufficient data estimating the prevalence of ocular involvement and the distinct clinical types in PV. In prospective, cross-sectional study on Iranian patients, 103 PV patients were designed to determine the prevalence, clinical types, and epidemiological trends of ocular involvement. Ocular involvement was present in 17 (16.5%) patients. Moreover, conjunctivitis was the most predominant type of ocular involvement (9/17, 52.9%), followed by erosion of the palpebral conjunctiva (7/17, 41.2%). Erosion of the bulbar conjunctiva was notable in only one patient (5.9%). The most frequently described symptoms were eye irritation (76.5%) and redness (76.5%).[5]

Scleritis is rarely reported in concurrence with PV. To the best of our knowledge, there are only a few reports describing the occurrence of scleritis during the course of PV.[7] Similar to our case, the reported case had a recalcitrant PV and persistent oral erosion and developed diffuse anterior nonnecrotizing scleritis only a few weeks after receiving rituximab therapy. The patient responded to oral indomethacin and prednisolone. In our case, the scleritis improved after the second dose of rituximab and prednisolone acetate eye drops.

The improvement in her ocular symptoms after the second dose of rituximab was concomitant with overall improvement of her pemphigus disease, which suggests that the scleritis is less likely a manifestation of PV. However, since both cases developed after rituximab therapy for PV, the role of rituximab in the development of scleritis cannot be ruled out. Although, up to our knowledge, there are no previous reports of scleritis induced by rituximab.

In conclusion, we present a case of diffuse anterior nonnecrotizing scleritis in concurrence with PV. Recognizing this association will hopefully guide ongoing immunosuppressive therapy.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initial s will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Broussard KC, Leung TG, Moradi A, Thorne JE, Fine JD. Autoimmune bullous diseases with skin and eye involvement: Cicatricial pemphigoid, pemphigus vulgaris, and pemphigus paraneoplastica. Clin Dermatol 2016;34:205-13.  Back to cited text no. 1
Mihai S, Sitaru C. Immunopathology and molecular diagnosis of autoimmune bullous diseases. J Cell Mol Med 2007;11:462-81.  Back to cited text no. 2
Sitaru C, Zillikens D. Mechanisms of blister induction by autoantibodies. Exp Dermatol 2005;14:861-75.  Back to cited text no. 3
Joly P, Litrowski N. Pemphigus group (vulgaris, vegetans, foliaceus, herpetiformis, brasiliensis). Clin Dermatol 2011;29:432-6.  Back to cited text no. 4
Akhyani M, Keshtkar-Jafari A, Chams-Davatchi C, Lajevardi V, Beigi S, Aghazadeh N, et al. Ocular involvement in pemphigus vulgaris. J Dermatol 2014;41:618-21.  Back to cited text no. 5
Mustafa MB, Porter SR, Smoller BR, Sitaru C. Oral mucosal manifestations of autoimmune skin diseases. Autoimmun Rev 2015;14:930-51.  Back to cited text no. 6
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. 7


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