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BRIEF COMMUNICATION
Year : 2013  |  Volume : 1  |  Issue : 3  |  Page : 157-159

Corneal keratin horn in a case of ocular cicatricial pemphigoid: A relentless disease


Department of Ophthalmology, Bharati Vidyapeeth Deemed University Medical College, Pune, India

Date of Submission25-Jan-2013
Date of Acceptance20-May-2013
Date of Web Publication23-Aug-2013

Correspondence Address:
Nandini Ramchandran
B-3/6, Salunke Vihar, Salunke Vihar Road, Pune
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2320-3897.116852

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  Abstract 

We report a rare case of progressive corneal blindness in a 65-year-old female, who was diagnosed as cicatricial pemphigoid, after almost three decades of suffering recurrent oral ulcerations. The left eye was affected earlier, and to a greater extent and severity. Subsequently, the disease progressed to complete corneal keratinization in the left eye. This case highlights the natural course of the disease, wherein the patient finally sought ophthalmic consultation for the recurrent appearance of a horn like growth in the left eye along with vision impairment due to cataractous change in the less affected right eye. At this time, patient had obvious features of ocular cicatricial pemphigoid (OCP) such as formation of multiple symblephara in the right eye and a hard dry growth in an immobile, blind left eye. To the best of our knowledge, corneal keratin horn formation has not been reported as a feature of OCP so far.

Keywords: Dry eye, keratin horn, ocular cicatricial pemphigoid, symblepharon


How to cite this article:
Ramchandran N, Puthran N, Muzumdar D. Corneal keratin horn in a case of ocular cicatricial pemphigoid: A relentless disease. J Clin Ophthalmol Res 2013;1:157-9

How to cite this URL:
Ramchandran N, Puthran N, Muzumdar D. Corneal keratin horn in a case of ocular cicatricial pemphigoid: A relentless disease. J Clin Ophthalmol Res [serial online] 2013 [cited 2019 Oct 18];1:157-9. Available from: http://www.jcor.in/text.asp?2013/1/3/157/116852

Ocular cicatricial pemphigoid (OCP), originally described by Cooper in 1858, [1] is a rare autoimmune disorder characterized by a progressive scarring and drying of the conjunctiva and cornea, and often results in gross visual disablement. It occurs in about 1:20,000-1: 40,000 population with a female:male ratio of 2:1. [2]

The visual prognosis is poor as the disease is often recalcitrant to medical treatment. Management of these cases presents a further challenge as patients are generally unsuited for any ocular surgery due to the severe dry eye and progressive keratinization of the entire ocular surface.


  Case Report Top


An elderly female complained of progressive impairment of vision in the left eye following a minor trauma, sustained about 30 years ago. It was accompanied by recurrent episodes of painful red eyes. Since the past 2 years, there was marked dryness of her left eye, with a dry white powdery discharge. She had also noticed a painless, slow growing, hard growth in her left eye. There was additional history of intolerance to several oral medications, in the form of recurrent dysphagia and oral ulcerations over the past 30 years. She had undergone multiple dental extractions in the past 2 years, following which the severity of the oral ulcerations had increased. A diagnosis of cicatricial pemphigoid was made at this time and confirmed by oral mucosal biopsy [Figure 1]. There was no history suggestive of involvement of any other mucosal sites. She was being treated with tab dapsone, 100 mg a day for the last 6 weeks, and tab prednisolone in tapering doses (starting with 60 mg and being tapered every 10 days. Patient was on 20 mg a day when she presented to us) for oral ulcers. She had not been advised any specific treatment for her ocular condition other than artificial tears off and on.
Figure 1: Histopathology findings compatible with cicatricial pemphigoid. Arrow marks submucosal bulla in oropharynx (H and E stain at 10×)

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Oral examination revealed several white, ulcerated, and congested mucosal lesions in the soft palate [Figure 2].
Figure 2: Arrows indicate multiple oropharyngeal ulcers

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Ophthalmic examination revealed a best corrected visual acuity of 6/18 in the right eye while the left eye vision was reduced to perception of light.

Right eye examination showed the presence of symblephera in the infero-temporal and supero-nasal fornices with submucosal scarring of the pretarsal conjunctiva of the upper lid [Figure 3]. There was no keratinization or significant dryness of the ocular surface. Corneal sensations were normal. The lens was cataractous. Posterior segment examination, intraocular pressure (IOP) reading and ocular motility tests were within normal limits.
Figure 3: Symblepharon of lower lid, Right side

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The left eye showed complete keratinization of the conjunctiva and cornea. There was a total symblepharon causing immobility of the eyeball and lids. There was distortion of both upper and lower lid margins. A keratin horn, of approximately 15 × 4-5 mm size, was seen arising from the center of the opaque cornea. No other details could be visualized [Figure 4]. The IOP could not be determined. The ultrasonography (USG) B scan of the left eye could not be done due to the protruding corneal horn.
Figure 4: Corneal keratin horn, Left eye

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Patient was taken up for removal of the brittle horn, which was easily shaved off. Artificial tears and topical 2% cyclosporine eye drops twice daily were prescribed for both eyes with continuation of systemically administered dapsone and steroids.

At 4 months follow-up, there are no fresh findings in the right eye. The left eye shows evidence of early reformation of the corneal keratin horn.


  Discussion Top


OCP is a rare autoimmune disorder, characterized by chronic, progressive, conjunctival inflammation and scarring. It is now regarded as one of the presentations of a disorder termed mucous membrane pemphigoid. [3]

The patient generally presents with symptoms of dry eye and recurrent conjunctivitis early in the disease. The disease is usually bilateral, although not necessarily symmetrical. OCP is classified into four stages, depending on severity of the clinical signs, which may range from chronic conjunctivitis and sub-epithelial fibrosis to corneal scarring and ankyloblepheron. [4],[5] The posterior segment is usually unaffected. Our patient suffered from both oral mucosal, as well as conjunctival inflammations for almost 30 years before she was diagnosed as a case of cicatricial pemphigoid following oral mucosal biopsy. Ocular involvement was asymmetric, the left eye being adversely affected to a greater extent than the right eye.

Histologically, a diffuse infiltration of the conjunctival basement membrane by macrophages, neutrophils, T cells, mast cells, and eosinophils, is seen. These cells, along with antibody/complement deposition, cause bullae formation and subsequent scarring of the conjunctiva. [2] Fibrosis of lacrimal ducts and loss of conjunctival goblet cells, ultimately, results in corneal keratinization and an insensitive eye. The disappearance of ocular pain in the left eye and the presence of normal vision in the right eye caused our patient to neglect her ocular problems.

Differential diagnoses include other causes of cicatricial conjunctivitis such as drug-induced cicatricial conjunctivitis, Steven Johnson's syndrome, etc., which are nonprogressive.

The goal of management is to suppress inflammation and prevent scarring. Immunomodulators, such as dapsone, are the first line of treatment. Systemic steroids may be used temporarily to suppress inflammation, but other immunosuppressive drugs are required in the long-term. These include azothiaprim, cyclosporine, and methotrexate. [5] Mycofenolate mofetil, immunoglobulins, and humanized monoclonal antibodies that bind to interleukin 2 on activated T cells have also been used. [6] Lubrication of the ocular surface with artificial tears may help to relieve the symptoms and allow smooth lid movements.

Our patient was given symptomatic treatment with lubricants over the past several years. She sought our consultation only after her right eye vision became impaired due to cataract formation. Cataract surgery in these patients should be by the clear corneal route. Ocular surgeries such as entropion surgery, surgical correction of symblepharon, reconstruction of the palpebral fornices, and implantation of keratoprosthesis in very advanced cases, should preferably be performed only after controlling the inflammation with peri-operative steroids. Other surgical procedures that may be required include epilation of misdirected eyelashes, punctal occlusion, and tarsorrhaphy for dry eye. In this patient, keratoplasty was not an option due to the dry eye. Patient is presently unwilling for a keratoprosthetic implant.

Although our patient has perception of light in the left eye, she is not a good candidate for either keratoplasty or any prosthetic devices due to the extreme keratinization and severe dryness. Enucleation of the eye with symblepharon release, and reconstruction of the fornices is also inadvisable due to the high risk of recurrence of the disease in the mucosal/amniotic membrane grafts.

The corneal horn seen in this patient is not a true horn as it is merely overgrowth of keratin and does not have any bony core. Generally, horns in humans are manifestations of hyperkeratosis and are seen as cutaneous outgrowths. [7] A search of literature, including Pubmed, has not revealed any previous reports of a corneal keratin horn in OCP

This case is presented for the depiction of the natural and relentless course of this rare autoimmune disease and the hitherto unreported occurrence of a corneal keratin horn.

 
  References Top

1.Benign mucosal pemhigoid, ocular pemphigoid: Essential shrinkage of the conjunctiva. In: Sir Stewart Duke Elder, editor. System of ophthalmology Vol. 8. Leighton, GB: Henry Kimpton Publishers; 1977. p. 502.  Back to cited text no. 1
    
2.Al-Ghoul A, Kim G, Dhaliwal DK. Ocular Cicatricial Pemphigoid/Mucosal Membrane Pemphigoid. In: Yanoff M, Duker JS, editors. Ophthalmology. Missouri, USA: Mosby; 2008. p. 410.  Back to cited text no. 2
    
3.Chan LS, Ahmed AR, Anhalt GJ, Bernauer W, Cooper KD, Elder MJ, et al. The first international consensus on mucous membrane pemphigoid: Definition, diagnostic criteria, pathogenic factors, medical treatment and prognostic indications. Arch Dermatol 2002;138:370-9.  Back to cited text no. 3
[PUBMED]    
4.Foster CS. Cicatricial Pemphigoid. Trans Am Ophthalmol Soc 1986;84:527-663.  Back to cited text no. 4
[PUBMED]    
5.Chang JH, McCluskey PJ. Ocular Cicatricial Pemphigoid: Manifestations and management. Curr Allergy Rep 2005;4:435-9.  Back to cited text no. 5
    
6.Foster CS, Hamam R, Letko E, Freeman J, Law SK, Rapuano CJ, et al. Ophthalmologic manifestations of cicatricial pemphigoid. Available from: htt p://emedicine.medscape.com/article/1191261-overview. [Medscape Updated 2011 Aug 23 rd ].  Back to cited text no. 6
    
7.Copcu E, Sivrioglu N, Culhaci N. Cutaneous horns: Are these lesions as innocent as they seem to be? World J Surg Oncol 2004;2:18.  Back to cited text no. 7
[PUBMED]    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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