Mucous membrane pemphigoid mimicking a corneal ulcer: A case report

Aarzoo Juneja; Athul S Puthalath; Himanshi Aggarwal; Neeti Gupta; Sanjeev K Mittal

doi:10.4103/ijo.IJO_2003_22

CASE REPORT

Year : 2023 | Volume : 3 | Issue : 1 | Page : 9-11

Mucous membrane pemphigoid mimicking a corneal ulcer: A case report

Aarzoo Juneja¹, Athul S Puthalath², Himanshi Aggarwal³, Neeti Gupta¹, Sanjeev K Mittal¹
¹ Department of Ophthalmology, AIIMS, Rishikesh, Uttarakhand, India
² Vitreous and Retina Services Aravind Eye Hospital, Madurai, Tamil Nadu, India
³ Ophthalmology, Former Junior Resident, AIIMS, Rishikesh, India

Date of Submission	17-Aug-2022
Date of Acceptance	18-Nov-2022
Date of Web Publication	20-Jan-2023

Correspondence Address:
Neeti Gupta
Department of Ophthalmology, AIIMS, Veerbhadra Road - 249 203, Rishikesh, Uttarakhand
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2003_22

Abstract

Mucous membrane pemphigoid (MMP) is a subepithelial blistering disorder affecting multiple mucous membranes, with ocular involvement in 70% of cases. A 50-year-old man had complaints of gradual and painless diminution of vision associated with redness since three months and had been diagnosed and managed as corneal ulcer outside. The patient did not improve clinically and presented to us with the above complaints. On systemic evaluation, he was found to have multiple skin lesions along with oral and genital ulcers. Punch biopsy of the lesions was suggestive of MMP. The patient was started on topical antibiotics and steroids along with systemic steroids and azathioprine. After one-month follow-up, there was improvement in both the ocular and skin lesions. MMP is a common cause of chronic cicatrizing conjunctivitis. One must be vigilant to diagnose and treat MMP before it presents as a blinding complication.

Keywords: Chronic cicatrizing conjunctivitis, corneal ulcer, mucous membrane pemphigoid, orogenital ulcer

How to cite this article:
Juneja A, Puthalath AS, Aggarwal H, Gupta N, Mittal SK. Mucous membrane pemphigoid mimicking a corneal ulcer: A case report. Indian J Ophthalmol Case Rep 2023;3:9-11

How to cite this URL:
Juneja A, Puthalath AS, Aggarwal H, Gupta N, Mittal SK. Mucous membrane pemphigoid mimicking a corneal ulcer: A case report. Indian J Ophthalmol Case Rep [serial online] 2023 [cited 2023 Jun 2];3:9-11. Available from: https://www.ijoreports.in/text.asp?2023/3/1/9/368202

Mucous membrane pemphigoid (MMP) is a systemic cicatrizing autoimmune subepithelial blistering disorder that predominantly affects the mucous membrane of the eye, nasal cavity, oropharynx, or genitalia. Ocular surface involvement may be variable such as dry eye, chronic conjunctivitis, and even corneal ulcer.^[1]

Case Report

A 50-year-old man presented with complaints of gradual painless diminution of vision associated with redness in the left eye (LE) for three months. There was no history of trauma, fever, or any recurrences in the past. He had earlier been seen by a local ophthalmologist and was diagnosed as a case of left eye corneal ulcer and started on topical medication, details of which were unknown to the patient. On ocular evaluation, the best-corrected visual acuity (BCVA) was 20/30 in the right eye (RE) and hand movement close to face (HMCF) with accurate light projection in all quadrants in the LE. Extraocular movements were full and free, and regurgitation test was negative in both eyes. On anterior segment examination with slit-lamp biomicroscope, the RE showed mild meibomitis, forniceal foreshortening (stage 2),^[2] keratinization in upper palpebral conjunctiva, blunting of caruncle and plica semilunaris along with arcus senilis and cataractous lens with nuclear sclerosis grade 3. LE examination revealed thickened eyelid margins, forniceal foreshortening (stage 3), and mixed conjunctival congestion, and a circular epithelial defect of diameter size 3 mm was seen in the paracentral region at 12–1 o'clock position on the cornea. There was also diffuse stromal edema, corneal thinning in central region at 3–4 o' clock position with hypopyon of 2 mm, and 360° vascularization [Figure 1]a and [Figure 1]b. Posterior segment examination of the RE was within normal limits, and LE fundus could not be visualized. Ultrasound B scan of LE revealed lens reverberations, clear vitreous cavity, normal optic nerve head, and attached retina. Systemic physical examination revealed oral ulcers and multiple skin lesions. Dermatological consultation revealed 4–5 aphthous-like ulcers in the right buccal mucosa near the angle of the mouth and single erosive lesion over the base of the penis [Figure 2].

Figure 1: (a) Slit-lamp biomicroscopy photograph of right eye showing forniceal foreshortening (red arrow) with conjunctival inflammation. (b) Slit-lamp biomicroscopy photograph of left eye showing corneal ulcer with descemetocele and hypopyon

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Figure 2: Oral mucosa showing multiple ulcers (star mark) and multiple genital ulcers (red arrow)

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On the basis of above clinical findings, a presumptive diagnosis of stage Ⅳ MMP was made.^[2] LE corneal scraping sent for microbiological assessment was unremarkable. Serological investigations were done including complete hemogram, random blood sugar levels and HbA1C, liver function test, kidney function test, urine routine and microscopy, HBsAg, and anti-HIV and anti-HCV antibodies which yielded normal results except for HbA1C, which was 7.3%. Punch biopsy of the oral ulcers was sent for histopathological and dermatopathological assessments. Histopathological examination showed subepidermal bullae with inflammatory reaction. Dermatopathological examination showed IgG and C3 linear immune deposits along the dermo-epidermal junction on direct immunofluorescence suggestive of MMP [Figure 3].

Figure 3: Section studied shows subepidermal bullae (black star) with intense lympho-plasmacytic infiltration in the sub epithelium. (H&E 40×)

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Cyanoacrylate glue and bandage contact lens was applied. Systemic steroids (prednisolone 1 mg/kg once a day), doxycycline antibiotic 100 mg twice a day, fortified topical antibiotics cefuroxime (2 hourly) and tobramycin (2 hourly), topical steroid (prednisolone acetate 1% 2 hourly), cycloplegic (homatropine 2% three times a day) and tear substitutes (carboxymethyl cellulose 0.5% 2 hourly) was started. The patient was also started on systemic immunosuppressants, tablet azathioprine 50 mg twice daily for four weeks, triamcinolone oral paste, and chlorhexidine mouth wash for oral lesions. He was kept on close observation, and after one month of follow-up RE ocular surface improved and the LE showed healing of descemetocele; hypopyon disappeared along with resolution of stromal edema and developed bony dry ocular surface with scarring of the cornea and conjunctiva [Figure 4]a and [Figure 4]b. Oral and penile lesions healed. The patient was kept on close follow-up every two weeks [Figure 5].

Figure 4: (a and b) RE ocular surface improved. LE showed bony dry ocular surface with scarring of the cornea and conjunctiva

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Figure 5: (a) and (b) showing healed oral and genital lesions

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Discussion

MMP is a heterogeneous group of chronic and autoimmune subepithelial blistering diseases that is characterized by deposition of immunoreactants (IgG, IgA, IgM, and/or others) along the basement membrane at the epithelial–subepithelial junction of mucous membranes. Ocular MMP usually presents as relapsing and remitting bilateral conjunctivitis.^[3],[4] Chronic conjunctival inflammation leads to progressive subepithelial fibrosis and tissue remodeling, while goblet cell destruction causes dry eye. Cicatrization also occurs in the adnexa, causing lagophthalmos and entropion, damaging the lid margins, and producing keratinization that result in corneal scarring and secondary infectious keratitis. Consequently, a corneal scarring induces neovascularization, conjunctivalization, and opacification, resulting in the marked loss of vision.^[5] Transforming growth factor beta (TGF-β), interleukin (IL)-4, IL-5, and IL-13 have an important role to play in the pathogenesis of MMP.^[6],[10]

MMP must be distinguished from other causes of chronic cicatrizing conjunctivitis. Bilaterality, extraocular mucous membrane involvement, occasional history of rash and blisters on the skin, associated systemic disease with progressive ocular inflammation are diagnostic of MMP. The main differential diagnosis includes infective adenoviral conjunctivitis, systemic lupus erythematosus, Wegener's granulomatosis, burns, acne rosacea, Stevens–Johnson syndrome, graft vs host Disease, and IgA Pemphigus and IgA Linear disease.^[1],[6] Our patient did not report any history of trauma to eye with vegetative matter, chemical injury, allergy, intake of systemic medications, previous eye surgery, or atopy. In the index case, biopsy from the oral mucosa showed IgG and C3 linear immune deposits along the dermo-epidermal junction, suggesting MMP.^[7] Preliminary baseline investigations including complete hemogram, random blood sugars, and renal and liver function tests were needed for initiating treatment. Complications such as dry eye and blepharoconjunctivitis were treated with preservative-free lubricants, daily eyelid hygiene, warm compressions, and topical antibiotics.^[8] Oral corticosteroids, topical antibiotics, lubricants agents, and cycloplegics were started in view of corneal ulcer with MMP. He was also started on the immunosuppressive agent, azathioprine.^[1] The patient showed improvement in ocular surface and in lesions of other mucous membranes.

MMP is the most common cause of cicatrizing conjunctivitis, with ocular involvement occurring to the order of 70%–75%. Keratitis is seen in approximately 1/4^th of patients having ocular features, with bilateral blindness in 20%.^[9] Ocular MMP is often diagnosed late due to non-specific early clinical manifestations and lack of clinically visible inflammation of the ocular surface with poor sensitivity of immunopathological diagnosis.

Conclusion

One must have an high index of suspicion in the evaluation of chronic conjunctivitis to prevent the blinding complications as a result of MMP. A clinician must not wait for clinical features to appear in other mucous membranes before initiating treatment as the eye may be the only site involved.

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 initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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