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Pemphigoid and pemphigus diseases as well as Stevens-Johnson syndrome present as vesiculobullous disorders of the skin and may additionally involve both the oral cavity and the ocular surface. Ocular involvement ranges from mild irritation and dry eye disease to chronic conjunctivitis, symblepharon, eyelid malposition, ocular surface scarring and severe visual loss. In addition to diagnostic assessments, ophthalmologists must treat the dry eye and meibomian gland dysfunction components of these diseases using a stepladder approach, including eyelid hygiene and lubricants. Topical anti-inflammatory therapy is used to treat acute inflammatory exacerbations of the ocular surface, but it cannot prevent scarring alone. Intralesional antimetabolite therapy can cause regression of conjunctival pathology in selected cases. Hence, patients with vesiculobullous disorders should be managed by a multidisciplinary team representing ophthalmology, dermatology, otolaryngology, oral medicine and pathology, internal medicine and intensive care. Systemic treatments including corticosteroids, azathioprine, cyclophosphamide, cyclosporine and mycophenolate mofetil help control inflammation. Intravenous immunoglobulins, plasmapheresis and targeted antibody therapy can be used in selected, severe and treatment-resistant cases. Local surgical management may include debridement of pseudomembranes, lysis of symblepharon, amniotic and mucous membrane grafting as well as reconstructive procedures. Prospective, multicentre, international studies are recommended to further support evidence-based practice.

A 4-month-old crossbred female dog presented with complaints of poor vision and odd appearance of the eyes. At the age of a month, the animal was found lying in a railway road in a very bad condition and was rescued from death. At that time, the patient was presented with severe bilateral ocular discharge, and from both eyes, dipterous fly larvae were extracted. The dog was given symptomatic treatment (deworming, topical and systemic antibiotics, nutritional support) but never regained a normal eye appearance. At the time of the initial presentation, it had severe vision impairment because of bilateral cicatricial symblepharon, total in the left eye and occupying 3/4 of the cornea in the right eye. Surgery was proposed to attempt a resolution. Superficial keratectomy was performed in both eyes. After a midline incision in the cornea, the conjunctiva was dissected away. The conjunctiva was apposed to and sutured to the limbus and secured in the upper and lower conjunctival fornix. Additionally, in the left eye, a lateral canthotomy and canthoplasty were performed to enlarge the size of the eyelid opening. The corneal epithelium healed without complications, the patient regained vision, and after 36 months of follow-up, no recurrences were observed. To the authors' knowledge, this is the first report of symblepharon secondary to ophthalmomyiasis externa in a dog.

Purpose: To describe a useful technique of surgical resection of dermolipomas. Methods: This is a retrospective, noncomparative and interventional case series. Sixteen eyes of 16 patients with dermolipomas and large areas of conjunctiva strongly adherent to the masses underwent surgical removal of the dermolipomas. The anterior portion of the mass, including closely adherent conjunctiva and pilosebaceous areas, was excised, and the conjunctival defects were covered with rotational conjunctival flaps from the superior or inferior bulbar conjunctiva. Results: The mean follow-up time was 12.5 months (range, 6–47 months). The cosmetic outcome was satisfactory in all 16 patients. There were no cases of reappearance of the mass, symblepharon or significant scar formation during follow-up. One patient developed blepharoptosis and another showed limitation of medial gaze postoperatively. Both were resolved spontaneously within 3 months. There were no other significant complications. Conclusion: Rotational conjunctival flap is a useful technique of conjunctival reconstruction following removal of large dermolipomas and the overlying epithelium.

Background Lichen planus is an autoimmune, inflammatory dermatosis of unknown cause that affects the skin and mucous membranes. Objective The aim of this study was to report the clinical features and response to therapy in a series of patients with ocular lichen planus. Methods A retrospective chart review was performed to identify patients with ocular lichen planus. Information about clinical presentation, treatment, and therapeutic response was extracted from the medical records. Results Eleven patients with ocular lichen planus were identified. The diagnosis was confirmed histologically for 10 patients. Nine patients were women. The average time from onset of ocular symptoms to diagnosis was 4.1 years. Eight patients had mucous membrane involvement at other sites. Disease was well controlled in eight patients. Conclusion Lichen planus should be considered in the differential diagnosis of cicatricial conjunctivitis, especially when severe lichen planus is noted at other sites.

Between February 1992 and March 1994, reconstruction of the fornices in 17 patients with extensive symblephara or lid fusion after most severe eye burns was performed with nasal mucosa from the inferior conchae as graft material. The time between accident and transplantation ranged from 2-64 months. All patients were followed for 6 to 31 months. Reconstruction of the fornices was achieved in 13 patients. Postoperative Schirmer-tests revealed markedly improved results. Impression cytology showed a persistence of goblet-cells and an excess of mucus. We have subsequently performed keratoplasties in 5 of these patients and are planning penetrating keratoplasties in a further 8 cases. In 4 patients, partial symblepharon formation recurred within 2-3 months after transplantation of nasal mucosa. The main advantage of nasal mucosa over buccal or labial mucosa may be the transplantation of intraepithelial goblet cells, leading to an improvement and stabilisation of the tear film.

This paper describes the use of amniotic membrane in the reconstructive surgical repair of generalized keratomalacia, ankyloblepharon, and after fibrous histiocytoma removal in two dogs and a cat. Case 1 was an 11-year-old female Yorkshire terrier with severe bullous keratomalacia in the left eye (OS). A frozen canine amniotic membrane graft and a third eyelid flap were applied. At day 80 postoperatively, only a mild scar and corneal vascularization were present. Case 2 was a 4-year-old female Siamese cat with symblepharon of both eyes after rhinotracheitis. Resection of the conjunctiva and frozen canine amniotic membrane transplant were performed. One month later, there was a little corneal scarring and corneal vascularization. Case 3 was a 6-year-old female terrier with a scleral and corneal mass at the 11-12 o'clock position (OS). Resection of the mass and amniotic membrane transplantation were performed. The mass was a fibrous histiocytoma localized to the sclera and cornea. This eye healed with mild conjunctivalization and no pigmentation. Amniotic membrane transplantation can be used as a method of reconstruction of the ocular surface with good repair of the cornea and minimal scarring in small animals.

Purpose: The surgical procedures used until now to rebuild the conjunctiva in patients affected by ocular cicatricial pemphigoid (OCP) have not demonstrated appreciable anatomical and functional success. This study reports the postoperative clinical and cytological outcomes of a human amniotic membrane transplantation (AMT) to rebuild the conjunctiva in a case of late stage OCP. Methods: We present a 75-year-old woman with a severe form of pemphigoid with entropion, trichiasis, symblepharon and blunting of the fornices, who underwent excision of the scarred and inflamed tissue covering the ocular surface and AMT. Results: Our data show an improvement of the ocular surface condition, with reacquired fornix depth, reduced inflammation and presence of goblet cells at each follow-up. Conclusion: Amniotic membrane transplantation was successful as a first step procedure to reduce inflammation and to rebuild a physiological conjunctival epithelium in late stage OCP.

Purpose Mucous membrane pemphigoid (MMP), previously known as cicatricial pemphigoid, is an autoimmune disease characterized by linear deposition of IgG and IgA along the epithelial basement membrane zone in direct immunofluorescence (DIF). DIF requires mucosal biopsy, which can worsen the condition by triggering an inflammatory response of the mucosa, especially the conjunctiva. We assess in vivo confocal microscopy (IVCM) for the detection of the pathognomonic basement membrane cleavage zone (BMCZ) in MMP Methods Six patients (4 M, 2 W), suffering from ocular and/or oral MMP were tested with IVCM, using the handheld Vivascope 3000. Three patients had ocular involvement, 2 oral involvement, and 1 had both. Results Mean age was 76 years old. Out of the 4 patients with ocular involvement, 2 patients had active conjunctivitis and 2 patients had cicatricial lesions. A BMCZ was identified in both patients with active conjunctivitis, while none was observed in those with cicatricial lesions. All 3 patients with oral involvement had desquamative gingivitis with marked BMCZ. Conclusion IVCM is non-invasive and reliable for the diagnosis of MMP. Conjunctival fornix biopsy is at high risk of symblepharon. These biopsies are often performed blindly as the pathological zones are barely visible clinically. IVCM enables the observation of the whole inflammatory surface of the conjunctiva and gum. To our knowledge, these are the first 2 cases of patients with conjunctival BMCZ clearly identified with IVCM.

In discussing the topic of systemic treat-ment of immunologic disease, it is im-portant to examine the treatment of ocu-lar allergic disorders, Type I hypersensit-ivity reactions. Surgeons who performcorneal transplants and treat externalocular diseases, commonly employ sys-temic medications in the treatment of im-mune-mediated ocular diseases that arenot Type I hypersensitivity diseases.There are four types of hypersensitivityreactions discussed by the Gell andCoombs and labeled as types I throughIV. Specifically, Type I is associated withIgE immunoglobulin, Type II is associ-ated with antigen-dependent cell cytotox-icity, Type III disease results from anti-gen-antibody complex deposition insmall blood vessels, and Type IV reac-tions are T cell-mediated diseases.Type I diseases are typified in the eyeby seasonal allergic conjunctivitis, vernalkeratoconjunctivitis, atopic keratocon-junctivitis, and giant papillary conjuncti-vitis. Type II hypersensitivity are seen inthe eye as Mooren’s ulceration and ocularcicatricial pemphigoid. Type III hyper-sensitivity reactions in the eye includerheumatoid arthritis, systemic lupus, po-lyarteritis nodosa, and Wegener’s Granu-lomatosis. Lastly, type IV reactions aretypified in the eye by corneal transplantrejection to endothelial cells as seen as aKhodadoust line with T lymphocytes.The differential diagnosis of allergicocular disease is important. Seasonal al-lergic conjunctivitis is the most commonocular allergy and is associated withother forms of allergic diseases, such asallergic rhinitis, sinusitis, and asthma. It’susually seasonal, occurring in the fall andspring, but may be panseasonal. Ad-ditionally, it can last for many years. Typ-ically symptoms and signs are itching,burning, redness, lid edema, and mucousdischarge.Treatment of seasonal allergic conjunc-tivitis usually consists of topical eyedropsincluding antihistamines, topical vaso-constrictors, combination antihistamineand vasoconstrictor medications, mastcell stabilizers, nonsteroidal anti-in-flammatory drugs, and topical steroids invery severe cases.Vernal keratoconjunctivitis is a bilat-eral recurrent disease seen in youngmales, typically between 5 and 14 years ofage with associated atopic disease. Uponeversion of the upper eyelid, one can seethe giant papillae or giant cobblestones.There is another subtype known as thelimbal form. One can observe aggre-gations of eosinophils in a gelatinousconfiguration at the limbus known asHorner’s dots or Trantas’ dots. Bothpresentations are the same disease, butthe findings occur in a different location.Children can present with ptosis be-cause the eyelids are very edematous andboggy, and hence heavy. Patients presentwith stringy, ropy mucous discharge, redeyes, cobblestone papillae of the uppereyelid and Trantas’ dots at the limbus.Additionally, patients may have cornealinvolvement with keratopathy as well asshield ulcerations. Patients complain ofmarked itching, tearing, burning, and as-sociated atopic diseases of sinusitis andasthma.Treatment of vernal keratoconjunc-tivitis is typically topical, with a combi-nation of antihistamines, mast cell stabil-izers, topical steroids in pulsed doses,nonsteroidal agents, and cyclosporin A,however, one must be careful when usingthese in children. Systemic treatment be-comes more important in this disease,with oral antihistamines and oral pred-nisone being added. As the diseases be-come more complex and threaten the cor-nea, the oral medications become ofparamount importance.Atopic keratoconjunctivitis is achronic disease. Unfortunately, many ofthese patients are diagnosed very late inlife. Patients have multiple complaintssuch as chronic itching, burning, redness,discomfort.Slit-lamp examination demonstratessubconjunctival scarring from thechronic inflammation; the meibomianglands may become totally occluded; andthe tear glands of Zeis and Wolfring maybecome scarred or nonfunctioning be-cause of the subconjunctival fibrosis. Pa-tients have chronically red eyes. As thedisease progresses, one may observe theformation of corneal abrasions becauseof the poor tear film.The ocular symptoms in these individ-uals are chronic itching, burning, mucousdischarge, photophobia, and tearing. Theocular signs are symblepharon forma-tion, corneal ulcerations, dry eye, and

The surgical treatment of lower lid entropion in ocular cicatricial pemphigoid has previously met with limited success and conventional techniques have in some cases caused disease progression. We have treated lower lid entropion in this condition with retractor plication, avoiding surgery to the conjunctiva in five patients (seven eyelids) over the past five years with successful correction of the condition and symptomatic improvement in all patients. It has not led to an acute exacerbation of the condition in any patient and we recommend it as the procedure of choice in the surgical management of entropion in this disorder.

Purpose Discussion of the evolution of a technique suggested by Jose I. Barraquer in 1947, later completed by Strampelli and adopted and modified by other corneal surgeons. The original procedure was based on the clinical observation that regeneration of the corneal epithelial cells is dependant on the state of the limbus. This explained the bad prognosis of chemical burns and severe limbus trauma. At present it is well known that the stem cells located in the corneal limbus are responsible for epithelial regeneration. Penetrating keratoplasty limited to the cornea does not supply stem cells. Methods The technique consists in obtaining and grafting limbus tissue, in form of an annular or partial conjunctivosclerocorneal limbal graft preferably from the fellow eye or a living donor, or an eye bank eye. A case of chemical burn operated in 1981 is presented to illustrate the original technique of Strampelli. To illustrate the modern approach a case of chemical trauma with superior and inferior symblepharon and total vascularized leucoma operated in 1999 (limbal donor graft)taking advantage of the modern advances available at present (improved instrumentation, pharmacological inhibition of homograft reaction)is shown. Results The results were encouraging.The patient operated in 1981 died in 1988 with good vision (0.6).In the case operated in 1999, three months after the limbal transplant, re-epithelization of the cornea was correct and an 8 mm penetrating keratoplasty combined with cataract extraction and IOL implantation was performed. Corrected vision 5 years postoperatively was 0.35. Conclusion Modern investigation and technology have converted a procedure based on surgical inspiration more than half a century ago into a valuable option in cases of irreversible damage of the corneal limbus.

Purpose: To describe a protocol for the reconstruction and replacement of the ocular surface (OS) Methods: Corneal epithelial stem cell deficiency (SCD) can result in a number of changes ranging from metaplastic corneal epithelium to complete corneal breakdown. Diagnosis of SCD is essentially clinical. Demonstration of goblet cells in corneal epithelium by impression cytology or biopsy is confirmatory. The extent of SCD is defined as unilateral or bilateral and partial (visual axis affected or spared) or total. Evaluation of the thickness and clarity of the corneal stroma underlying the fibrovascular pannus, the visual potential by electrophysiological tests, the intraocular pressure, workup of the patient for imunosuppession and HLA typing of the patient and potential living related donors are also required before embarking on treatment. Results: For Partial SCD where the visual axis is involved, Sequential Sector Conjunctival Epitheliectomy is indicated. For total unilateral cases auto limbal transplantation is the treatment of choice and for total bilateral cases allo-limbal, cadaver or living related donor transplants are indicated. Amniotic membrane as a patch or graft and ex-vivo expanded cell sheets can also be used. Associated lid abnormalities, symblepharon (buccal mucosa graft), glaucoma and cataract should ideally be dealt with prior to undertaking OS restorative surgery. When a corneal graft is needed it is best done as a second procedure after the ocular surface has been restored. When an intumescent cataract is associated with raised pressure, corneal gafting may become a necessity if a dense fibravascular pannus or corneal scar precludes visualisation of the interior of the eye. Longterm immunosuppession is usually required Conclusions: This approach provides the best chance of success

Therapeutic contact lenses are a rewarding area of optometric practice. Within Sunderland Eye Infirmary a diversity of ocular conditions present, creating a wide range of challenges. A variety of case histories will be discussed. A prosthetic scleral shell was made for a chemical burn injury which presented with symblepharon. A traumatic cataract (caused by a flying contact lens lid) was extracted and an aphakic hydrogel contact lens with an opaque iris print and clear pupil was required to maintain vision and decrease symptoms of glare. Two cases of perforating injury resulting in opaque corneas are shown. In one case the lens was ordered from a trial set, the other prosthetic contact lens was hand-painted to match a photograph sent to the manufacturer. A hydrogel occlusive pupil contact lens was used to cover a dense white inoperable cataract caused by recurrent chronic uveitis in rheumatoid arthritis. This type of contact lens is also used for diplopia therapy.