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Purpose The purpose of the report was to describe the first successful tarsal fracture surgery in a 1-year-old boy diagnosed with cicatricial ocular pemphigoid whose visual and psychomotor development were notably limited. Methods We present the case of a 1-year-old boy diagnosed with mucous membrane pemphigoid by biopsy who was treated with rituximab (375 mg/m2 intravenous infusion at 2-week interval administered twice) and stable with oral dapsone (2 mg·kg-1·d-1). His eyelid cicatricial entropion and trichiasis in both eyes prevented him from opening his eyes, impeding visual development. After 1 year of clinical stability, we performed a tarsal fracture procedure in both eyes to restore eyelid anatomy and functionality, with the aim to prevent an inflammatory reaction, administrating intravenous dexamethasone before and after surgery. Results The intervention was successfully performed without postoperative complications. Excellent anatomic and functional results allowed him to develop normally in his daily life the first week after surgery. He is currently taking oral dapsone (2 mg·kg-1·d-1) as a maintenance treatment to stop the progression of the disease. Conclusions Tarsal fracture surgery may be considered part of the treatment in pediatric patients with stable ocular cicatricial pemphigoid presenting with severe entropion and trichiasis.

PURPOSE Ocular cicatricial pemphigoid (OCP) can lead to devastating ocular complications without prompt treatment. A number of immunomodulatory agents have been attempted with varying success. The objective of this study was to evaluate the use of rituximab as an adjuvant to immunomodulatory treatments (IMTs) in refractory OCP. METHODS The clinical records of 14 patients with treatment refractory OCP treated with rituximab as monotherapy or in combination with IMTs were retrospectively reviewed, with a focus on demographics, treatments prerituximab and postrituximab, total number of rituximab infusions, response to treatment, and ocular outcomes including staging and best-corrected visual acuity. RESULTS Thirteen patients (92.9%) achieved a complete response with rituximab over a mean period of 4.3 months. The average sustained complete response time in those without relapse was 29.7 months. Five patients relapsed over a mean period of 15 months, 2 of whom were able to regain control over a mean of 2.5 months with additional rituximab treatments. At the final evaluation, rituximab-based therapy improved ocular outcomes in OCP by stabilizing Foster staging and preventing the deterioration of best-corrected visual acuity in 72% of eyes. In total, 90% of eyes with Foster stages 3 or less did not progress. All patients were able to decrease IMT dosage and/or transition to less potent adjuvant treatments. CONCLUSIONS The consideration of rituximab earlier in treatment of OCP as a rescue and/or maintenance therapy could result in an earlier arrest of disease progression, resulting in preservation of patients' vision, and enable tapering of adjuvant IMT.

Stevens-Johnson syndrome, ocular cicatricial pemphigoid, and severe thermal or chemical injury are considered severe ocular surface disorders (OSDs) because they affect the entire ocular surface, including corneal and conjunctival epithelial stem cells. In patients with severe OSDs, the long-term prognosis for limbal transplantation is poor, and the related corneal opacity and cicatrization lead to devastating visual impairment. To date, there is no standardized treatment to improve vision in cases with severe OSD. Investigating novel treatment methods for severe OSDs, our group began cultivated oral mucosal epithelial transplantation in 2002 and developed a limbal-supported rigid-type contact lens that can be applied as a nonsurgical treatment. When used in combination, these treatment methods make it possible to successfully restore vision in cases with severe OSDs.

PURPOSE To describe a novel surgical technique using the Boston Keratoprosthesis (KPro) type I in a patient with advanced ocular cicatricial pemphigoid (OCP) using oral mucosa for covering the prosthesis. METHODS We present the case of an 85-year-old man previously diagnosed with type 2 diabetes and advanced OCP nonresponsive to immunosuppressive treatment, whose best-corrected visual acuity was light perception and projection in both eyes. After examination, Boston KPro type I in the right eye was contemplated because osteo-odonto KPro and Tibial bone KPro were not feasible because of the patient's osteoporosis and edentulism. Reconstruction of the ocular surface was first performed using oral mucosa to release the symblepharon and try to deep the fornices. Three months later, the oral mucosa was lifted, and the Boston KPro type I was implanted using the patient's own cornea. Then, a modification of the standard surgical technique was carried out, replacing the use of contact lens for covering the prosthesis with an oral mucosa graft with a central trephination as an alternative option in fornix foreshortening cases. RESULTS After 11 months, visual acuity was stable to 0.2 decimal. No postoperative complications have been encountered, and prosthesis was in place. CONCLUSIONS The surgical technique of transmucosal Boston KPro type I may be considered a surgical alternative in patients with advanced OCP who present with severe fornix foreshortening, where osteo-odonto KPro or Tibial bone KPro cannot be performed due to osteoporosis or edentulism or when the Boston KPro type II is not readily available.

Ocular cicatricial pemphigoid (OCP) is a rare systemic autoimmune disease and a potentially blinding subepithelial blistering disorder. The purpose of this study was to describe the clinical spectrum of the disease and to assess the efficacy and safety of immunosuppressive agents in a cohort of patients with OCP.We conducted a monocentric retrospective cross-sectional cohort study of all unselected consecutive patients diagnosed with progressive OCP. Ocular and extra ophthalmological involvement as well as histological findings were gathered. Other outcomes were exposures to immunosuppressive agents defined by the use of a particular treatment. For each exposure, success in controlling ocular inflammation was graded as a complete response, response, or failure. Relapses and adverse events (AE) were also recorded.Seventeen patients (34 affected eyes), 35% of whom were women, were included, with an age at diagnosis of 75 ± 11 years. Corneal involvement was diagnosed in 30 of 34 eyes, and 22 of 34 eyes had progressive fibrosing conjunctival involvement. Sixty-two exposures to immunosuppressive agents or biologics were recorded: dapsone, n = 26; mycophenolate mofetil, n = 6; azathioprine, n = 4; cyclophosphamide, n = 10; rituximab, n = 14; and intravenous immunoglobulin, n = 2. Rates of response and of complete response achievement during the first 3 months were 84% and 45%, respectively. Response rates were 100%, 100%, 86%, 85%, and 80% for intravenous immunoglobulin, mycophenolate mofetil, rituximab, dapsone, and cyclophosphamide, respectively. Thirteen percent of those drugs were discontinued because of an adverse event in 4 patients.This study describes the efficacy of immunosuppressants or biologics with an acceptable safety profile for OCP.

Purpose The purpose of this study was to report the clinical course and outcome of patients with refractory ocular mucous membrane pemphigoid (MMP) treated by repository corticotropin injection (RCI). Methods Patients with biopsy-proven ocular MMP treated with RCI from 3 tertiary medical centers were evaluated. Medical records between January 2013 and January 2021 were reviewed and deidentified to retrieve relevant disease-related data. Primary outcome measures included conjunctival inflammatory activity, change in Foster clinical conjunctival scarring staging after RCI treatment, and the development of ocular and systemic complications. Results Included were 15 patients (10 women and 5 men; 36-95 yrs of age) with a mean follow-up of 4.5 years. Most of the patients (80%) had Foster stage 3 at presentation, and all patients had active MMP. Each patient had failed to respond to at least 1 immunomodulatory drug during the follow-up, and 9 (60%) patients had treatment failure of at least 2 other agents before the use of RCI. The mean duration of RCI treatment was 21 months (range, 3-54 mo). Foster stage did not change in any of the 15 patients at the last follow-up. Nine patients continued RCI therapy at the last follow-up, and in all of them, the disease activity of MMP was well controlled. No serious adverse events because of RCI were documented during the follow-up in any treated patient. Conclusions RCI may serve as an alternative or an adjunctive treatment in patients with severe and refractory ocular MMP. Treatment with RCI seems to be safe and well-tolerated.

Purpose To evaluate the presence of atopy in patients with ocular cicatricial pemphigoid (OCP). Method Patient encounters between August 2005 and November 2016 at the Massachusetts Eye Research and Surgery Institute (MERSI) were searched to identify those with biopsy-proven OCP who had concurrent evidence of atopy. Results There were 230 patients with biopsy-proven OCP. Thirty-three of them were found to have clinical symptoms of atopy (asthma, hay fever, and eczema) and of these, 23 had evidence of atopy in their conjunctival biopsy specimens. All patients were administered immunomodulatory therapy for treatment of their OCP with 20 patients requiring additional antiallergy treatment to control residual atopic ocular symptoms. Among patients who used antiallergy medications, 80% showed improvement in residual symptoms. Rituximab and/or intravenous immunoglobulin is a preferred OCP medication for patients with OCP with some evidence of atopy. Conclusions Clinicians should consider the coexistence of atopy in patients with OCP, especially in those with persistent symptoms after initiation of immunomodulatory therapy.

To investigate the efficacy and safety of transplantation with biomaterial-free cultured oral mucosal epithelial cell sheets (COMECs) for ocular reconstruction in subjects with total limbal stem cell deficiency.A prospective clinical trial (NCT02149732) was conducted in 8 subjects with total limbal stem cell deficiency after approval from the institutional review board of Seoul National University Hospital (H-0707-043-213) and the Ministry of Food and Drug Safety of Korea. COMECs were prepared in a culture system without the use of any temperature-sensitive polymers or carriers. The COMECs were transplanted without suture fixation. Four subjects underwent penetrating keratoplasty after stabilization of the COMEC transplant. Stable epithelialization, changes in visual acuity, and postoperative complications were evaluated for 6 months. Corneal cytokeratins (K) of 4 subjects who underwent penetrating keratoplasty were stained with an immunofluorescent agent.The ocular surface was successfully reconstructed in 6 eyes. Complete stable epithelialization was achieved within a mean of 53.6 days. Visual improvement (≥2 lines) was achieved in 62.5% of the eyes. K12 (corneal phenotype), K4, and K13 (mucosal phenotype) were well expressed in grafts after keratoplasty, whereas K1, K8, and K19 were barely expressed. No ocular infections, local tumor formation, or remarkable systemic complications were observed. Ocular reconstruction using COMECs failed in 2 eyes, which had full symblepharon in 4 quadrants.Transplanting biomaterial-free COMECs seems to be an efficient and safe procedure to reconstruct the ocular surface in patients who are completely limbal stem cell deficient without a full symblepharon.

PURPOSE To describe the morphological features of the corneal epithelial layers, subbasal nerve plexus, stroma, and endothelium in patients with mucous membrane pemphigoid (MMP) as shown by in vivo confocal microscopy (IVCM). METHODS Central corneal images were captured from 10 healthy age-matched control eyes and 30 eyes with clinically diagnosed MMP by in vivo laser scanning confocal microscopy (HRT III RCM). Morphological changes of the corneal epithelial layers, stroma, and endothelium, characteristics of corneal nerves, and presence of inflammatory dendritic cells (DCs) were evaluated. RESULTS Images obtained by IVCM from 40 eyes were analyzed. The eyes with MMP were divided into 2 groups based on clinical staging: 16 eyes with end-stage MMP and 14 eyes with non-end-stage MMP. Compared with controls, IVCM in eyes with end-stage MMP displayed severe conjunctivalization and neovascularization of the cornea, with otherwise limited identifiable cellular or structural elements. Those with non-end-stage MMP showed metaplasia of the corneal epithelial layers, presence of hyperreflective cells similar to conjunctival cells, intraepithelial defects, fibrosis of anterior stroma, and hyperreflective endothelial deposits. Images of the subbasal nerve plexus demonstrate significant reduction in density (1251.3 ± 806.9 μm/frame vs. 2688.8 ± 607.33 μm/frame, P < 0.001), increased tortuosity (2.76 ± 0.6 vs. 2.3 ± 0.42, not significant), decreased reflectivity (2.73 ± 0.4 vs. 3.46 ± 0.52, P < 0.01), and increased density of DCs (115 ± 88 cells/mm vs. 43.9 ± 28.14 cells/mm, P < 0.05) in MMP-affected eyes compared with controls. CONCLUSIONS IVCM reveals profound and variable microstructural changes in the corneas of patients with MMP compared with normal controls. Our study demonstrated decreased corneal nerve density and elevated DC density in eyes with non-end-stage MMP compared with normal controls. Frequent scarring, conjunctivalization, and neovascularization observed in eyes with end-stage MMP preclude recognition by IVCM of the morphologic architecture of the corneal layers. Our findings suggest implications for using IVCM to evaluate and monitor patients with MMP.

PURPOSE To describe an unusual cicatrizing manifestation of periocular amyloid and the utility of adjuvant antimetabolite therapy. METHODS This is a case report of a 49-year old woman with progressive bilateral upper lid ptosis, who was found to have bilateral inferior forniceal masses with thickening of the conjunctiva. Conjunctival and eyelid biopsies showed evidence of amyloid deposition. Systemic evaluation did not reveal any evidence of systemic amyloidosis. Her blepharoptosis continued to deteriorate, and she underwent bilateral sequential upper lid ptosis correction surgery and debulking procedures. Subsequently, she developed severe and progressive cicatricial conjunctivitis with extensive symblepharon formation in all 4 fornices and restriction of ocular motility, simulating a clinical picture of ocular mucous membrane pemphigoid. RESULTS She was treated surgically by division of symblepharon augmented with application of topical mitomycin C intraoperatively. This has been partially successful in freeing up differential movement between her lid and her eye and consequently improving eye protection and lubrication. CONCLUSIONS Pseudopemphigoid represents a rare manifestation of periocular amyloid. Risks and benefits of surgical procedures in this context need to be carefully weighed to minimize the risk of secondary complications. Topical mitomycin C may be considered as a relatively safe adjunct in managing the cicatricial component of the disease.

To evaluate the clinical outcomes of mycophenolate mofetil (MMF) treatment of mucous membrane pemphigoid (MMP).This is a retrospective analysis of consecutive patients with clinical MMP seen in the Ocular Surface Disease Clinic at the Wills Eye Institute, between January 1, 2004, and December 31, 2010, treated with MMF. The main outcomes measured were control of inflammation and discontinuation of MMF.A total of 23 MMP patients taking MMF were identified. The median age of the MMF-treated patients was 77.0 years. Eleven of the 23 patients (47.8%) had biopsy-proven MMP. All patients were at least Foster grading system stage 2, with most stage 3 or 4. Eight patients (34.8%) failed previous treatments with dapsone, methotrexate, prednisone, azathioprine, cyclophosphamide, or 6-mercaptopurine. The average duration of MMF treatment was 23.32 ± 33.17 months (range 1-124.83 months, median 7.4 months). Of the 23 patients with MMP, control of inflammation was achieved with MMF within 3 months for 56.5% [95% confidence interval (CI) 54.5-59.6], within 6 months for 69.6% (95% CI 65.2-76.6), and within 12 months for 82.6% (95% CI 75.3-92.4) of the patients. Nineteen patients (82.4%) achieved control of inflammation, with 16 of the 19 (84.2%) achieving control of inflammation with MMF as monotherapy. Fifteen patients were treated with MMF as initial therapy. Twenty-one percent of patients (5 of 23) were taken off MMF for failure of inflammatory control (4) or an allergic reaction (1).Treatment of MMP with MMF in this uncontrolled case series resulted in control of inflammation in the majority of patients with minimal side effects. Our data support consideration of MMF as an initial treatment option for active ocular MMP.

PURPOSE Despite the availability of systemic immunosuppressants, cicatricial conjunctivitis (CC) remains a potentially blinding ocular surface disease. We aim to describe the combined use of rituximab (RTX) and intravenous immunoglobulin (IVIg) for severe recalcitrant autoimmune CC. METHODS In this single-center retrospective interventional case series with follow-up between 32 and 65 months, 3 cases with mucous membrane pemphigoid (patients 1-3) and 1 case with linear IgA disease (patient 4) were included. Initial conventional immunosuppressive therapy regimens included systemic steroids, dapsone, and mycophenolate. At the time of initiation of RTX and IVIg, all patients had only one eye with good visual acuity or good visual potential. Treatment included 1 to 2 cycles of RTX (1000 mg twice at an interval of 2 weeks apart), and 2 to 9 monthly courses of IVIg (2 g/kg over 3 days). Outcome measures were blindness, as defined by best spectacle-corrected visual acuity

To determine the influence of topical vancomycin prophylaxis on the incidence of bacterial endophthalmitis in eyes with a Boston Keratoprosthesis (KPro).A retrospective chart review was performed for 255 eyes of 231 patients who received a KPro between March 1990 and December 2006. Preoperative diagnoses were burn, ocular cicatricial pemphigoid (OCP), Stevens-Johnson Syndrome (SJS), and graft failure/other. Patients used topical antibiotic prophylaxis for the duration of the KPro: polymyxin-trimethoprim or a quinolone in the 1990s, or a quinolone with or without vancomycin beginning in late 1999. For each KPro eye, the follow-up interval was divided into months on or off vancomycin (vancomycin versus no-vancomycin group). The incidence of endophthalmitis was calculated with Kaplan-Meier survival curves.The 255 eyes were followed for 673.6 patient-years (mean, 2.64 years; range, 1 week to 13 years). There were 18 cases of bacterial endophthalmitis; 17 occurred at least 6 weeks postoperatively (range, 1.5 to 46 months). Gram-positive cocci caused over 80% of cases. Only 1 case, due to an atypical mycobacterium, occurred in a patient using vancomycin.The incidence of bacterial endophthalmitis was lower in the vancomycin group than in the no-vancomycin group: 0.35% versus 4.13% per patient-year (P = 0.001). It was also lower in SJS eyes using vancomycin versus no vancomycin: 1.76% versus 18.39% per patient-year (P = 0.009). In eyes with preoperative diagnoses of burn, OCP, or graft failure/other, the incidence in the vancomycin group was zero.Topical vancomycin plus a quinolone is effective in preventing bacterial endophthalmitis in KPro eyes.

PURPOSE To evaluate the long-term clinical efficacy of the Seoul-type keratoprosthesis (S-KPro). METHODS S-KPros were implanted into 4 unsighted and 5 sighted eyes in 9 patients: 6 patients were diagnosed with Stevens-Johnson syndrome, 2 had chemical burns, and 1 suffered from ocular pemphigoid. The preoperative visual acuity ranged from light perception to hand motion. The average follow-up period was 62.8 months. We evaluated several clinical factors, including visual acuity, visual field, number of additional grafting procedures, number of capsulotomy procedures, and the interval between retinal detachment and skirt exposure. RESULTS The S-KPro showed anatomic success for an average of 62.8 months in 66.7% of the eyes. The average visual acuity preservation time was 31.6 months. Localized glaucomatous visual field defect was not found in any of the sighted patients; however, diffuse visual field constriction was observed after long-term follow-up. Average time of skirt exposure and mean number of additional grafting procedures were 12.9 months and 2.44, respectively. Retinal detachments were developed in all the patients at a mean time interval of 2 months after S-KPro exchange. CONCLUSIONS S-KPro achieved visual rehabilitation for an average of 31.6 months with long-term anatomic stability in patients with severe intractable ocular surface disease.

Purpose: This study was designed to report a case of biopsy-proven mucous membrane pemphigoid with severe bronchial involvement in a young woman. Methods: Case report of a 17-year-old girl who presented with worsening dyspnea, skin rash, and bilateral ocular injection, symblepharon, and fornix foreshortening. Conjunctival, skin, and bronchial biopsies were performed along with imaging and serological tests in an effort to establish a diagnosis for this unusual constellation of findings. The surprising occurrence of a cerebrovascular accident during her hospitalization also prompted a search for a concurrent coagulation disorder. Results: Immunofluorescence studies of conjunctival, skin, and bronchial tissue specimens revealed deposition of multiple antibody classes at the basement membrane zone. The patient also possessed circulating basement membrane zone antibodies in her serum and a significant titer of antiphospholipid antibodies. She underwent dilation and stent placement for subglottic tracheal and left bronchial stenosis and was treated with immunosuppressive agents. After a favorable initial response, the patient experienced progressive bronchial stenosis and respiratory compromise, culminating in her death from bronchospasm and cardiopulmonary arrest. Conclusion: To our knowledge, this is the first report of mucous membrane pemphigoid involving the lower airways that was confirmed by immunofluorescence analysis. It highlights the potentially lethal, systemic nature of mucous membrane pemphigoid and underscores the need to question patients about symptoms of respiratory dysfunction.

Purpose To report a case of mucous membrane pemphigoid (MMP) occurring in childhood with exclusively ocular involvement. Methods Case report. Results A 12-year-old girl presented with mild bilateral conjunctival cicatrization, symblepharon, and peripheral inflammatory keratitis. No other mucosal or cutaneous involvement was present. The findings of an initial conjunctival biopsy with immunofluorescence study were negative for MMP. After a few recurrences of mild corneal and conjunctival inflammation, a second conjunctival biopsy was performed at a different site in the same eye, 30 months later. Only then was the diagnosis of MMP confirmed using positive direct immunofluorescence. The results of a systemic investigation for autoimmune vasculitis were negative. Therapy with dapsone was introduced, and a good response was obtained. Conclusions MMP is an extremely rare condition in children. This case is the second one reported with exclusively ocular involvement. Despite its rarity in the pediatric age group, MMP should be considered in the differential diagnosis of chronic conjunctival inflammation and fibrosis. Furthermore, a single negative biopsy should not exclude this diagnosis in a suspected clinical case, even in children. When a high degree of suspicion is present, a repeat biopsy may prove useful.

PURPOSE To report the case of a patient developing fungal keratitis in the context of uncontrolled ocular cicatricial pemphigoid (OCP), which, despite intravenous immunoglobulin (IVIg) and other immunomodulatory therapy, progressed to end-stage pemphigoid, with corneal opacification, ankyloblepharon, and xerosis. Keratoprosthesis (KPro) restored functional vision for the patient. METHODS A 39-year-old man presented with uncontrolled CP and corneal ulcer in the left eye. Conjunctival biopsy diagnosed OCP; corneal scraping and biopsy diagnosed the cause of the corneal ulcer. OCP was treated with systemic steroids, immunosuppressive drugs, and IVIg. Visual rehabilitation was accomplished with Ahmed valve and a type II Dohlman KPro. RESULTS Immunohistology of the biopsied conjunctiva showed IgG at the epithelial basement membrane zone, confirming the clinical diagnosis of OCP. Microbiologic studies of the corneal biopsy specimen were negative for Acanthamoeba and herpes but positive for Aspergillus niger. The patient's keratomycosis resolved with topical antifungal therapy. Treatment with Dapsone, intravenous-pulse steroid, oral cyclophosphamide, and intravenous immunoglobulin (IVIg) failed to control the OCP, with resultant complete conjunctivization of the cornea. Keratoprosthesis improved the patient's visual acuity from hand movements to 20/20. CONCLUSIONS Patients with uncontrolled OCP are at increased risk of corneal infection. The difficulty in diagnosing keratomycosis and the relatively rare occurrence of OCP explain the uniqueness of our reported case. OCP may progress to "end-stage" disease despite therapy. Keratoprosthesis can restore vision in selected otherwise seemingly hopeless cases.

Purpose: To describe a case of peripheral ulcerative keratitis (PUK) that developed in the immediate postoperative period after uncomplicated cataract surgery in a patient with ocular cicatricial pemphigoid. Methods: Single interventional case report. An elderly white woman with stable ocular cicatricial pemphigoid underwent an uncomplicated clear corneal phacoemulsification procedure in the left eye. In the immediate postoperative period, PUK developed adjacent to the corneal wound in the left eye. Results: The PUK was successfully treated with a bandage contact lens to the left eye, optimizing the ocular surface with punctal plugs and topical and systemic steroid therapy. After a 7-week course of tapering topical and systemic steroids, the PUK completely resolved. There was no further flare-up over a 12-month period. Conclusions: PUK can develop after clear corneal cataract surgery in patients with stable ocular cicatricial pemphigoid. Perioperative immunosuppression should be considered to minimize the chance of PUK developing in such cases

Ocular cicatricial pemphigoid (OCP) is a relatively rare autoimmune disease affecting elderly patients and causing severe symptoms that may culminate in blindness. Treatment is based on immunosuppression, but optimal regimens have not been established.A prospective unmasked case series of all patients with severe OCP who gave consent and completed treatment and ≥6 months of follow-up in the cornea and immunomodulation outpatient clinics of an academic hospital. Monthly pulses of intravenous cyclophosphamide (IVC; 500 mg) were administered with ondansetron and adjusted according to response.Over 14 years, 13 patients (median age 77 years) met inclusion criteria. All had bilateral OCP (2 eyes were previously blind), but extraocular involvement was rare (1/13). Three to 28 pulses were given, and the patients were followed up for a median of 32 (range, 6-167) months. Remission of inflammation in both eyes was achieved in 12 patients (92%). Vision improved in 5 of 13 patients, stabilized in another 5 (combined, 77%), and worsened in only 3 patients. One patient's condition flared up during treatment that responded to steroids and increasing IVC frequency. In 4 patients and 6 eyes (25%), cicatrization progressed (usually, Foster stages 1-3). Two late relapses occurred and responded to retreatment. IVC was generally well tolerated, although nausea led to modification in 2 patients. One patient developed candida keratitis.Compared with other treatment modalities, low-dose monthly pulse IVC is found to be a relatively safe, simple, and usually effective alternative immunosuppressive treatment in severe OCP.

PURPOSE Ocular mucous membrane pemphigoid (MMP) is known to be rapidly progressive in younger patients and affects both eyes. We are aware of only one other reported case of unilateral ocular MMP. The case presented in this study is unique in that it demonstrates genuinely unilateral ocular MMP in a younger patient. METHODS We report a case of a 50-year-old man who presented with a 3-month history of left eye redness, irritation, and mild discharge. He also suffered from mouth ulcers, skin lesions, and recurrent nose bleeds secondary to nasal mucosal lesions. RESULTS Examination revealed unilateral lid granulomas and cicatricial conjunctivitis in his left eye. There were no abnormal findings in his right eye. Biopsy of the lesions showed nonspecific inflammation with positive immunofluorescence for immunoglobulin G and C3 on epithelial basement membranes of conjunctiva, buccal mucosa, and skin, which were consistent with MMP. He was treated with immunosuppression and had no disease progression at 12-month follow-up. CONCLUSIONS This represents a rare case of unilateral nonprogressive ocular MMP in a younger patient. Histological analysis and immunofluorescence testing excluded a neoplastic process and confirmed the diagnosis. This case demonstrates that the presentation of unilateral eyelid pyogenic granulomas should include ocular MMP in the differential diagnosis once a neoplastic process has been excluded.

To determine the efficacy of a one-piece lamellar keratolimbal graft for the treatment of ocular surface disorders involving the limbus and cornea.A retrospective study of a case series including eight patients with either Stevens-Johnson syndrome (four patients), ocular cicatricial pemphigoid (three patients), or familial subepithelial amyloidosis of the cornea (one case) was performed with a mean follow-up period of 18 months after surgery.Of the eight cases, three patients retained a corneal epithelial appearance upon their latest visit. No statistically significant difference was observed between preoperative visual acuity (0.013) and postoperative acuity (0.082). Stromal neovascularization was observed in all cases. Seven of eight cases experienced persistent epithelial defects (PED), four of which evolved secondary infectious ulcers. Two cases required further surgical intervention to treat PED or infectious ulcers. None of the cases developed glaucoma.A one-piece keratolimbal graft does not seem to offer any advantage over a two-piece limbal and corneal graft in the treatment of severe ocular surface disorders involving the limbus and cornea.

PURPOSE To report the favorable outcome of a patient with severe ocular cicatricial pemphigoid, which was refractory to conventional treatment but recovered after intravenous immunoglobulin therapy. METHODS A case report. RESULTS Conventional therapy failed to control the ocular symptoms of this patient, which are typical of the disease. However, the patient improved remarkably in response to pulsed intravenous human immunoglobulin therapy. CONCLUSIONS We achieved stabilization of the disease around the seventh intravenous immunoglobulin cycle, thus contributing to satisfactory postoperative evolution after limbal allograft transplantation and facilitating the performance of intraocular surgical procedures, such as phacoemulsification, with improvement in best-corrected visual acuity.

To assess the pathogenesis, symptomatology, and severity of rheumatoid arthritis (RA) in patient's with concomitant ocular cicatricial pemphigoid (OCP).We retrospectively analyzed the charts of eight patients seen at a single institution between the years 1972 and 1997 with concomitant RA and OCP. Patients with OCP secondary to medical therapy, radiation, or chemical burns, or Stevens-Johnson syndrome were excluded.The female-to-male ratio was 7:1. All patients had positive serum rheumatoid factors and immunohistochemical confirmation of OCP. The mean number of years of RA prior to OCP diagnosis was 19. The number of patients with stage II OCP was three of eight (37.5%). The number of patients with stage III OCP was five of eight (62.5%). All patients had radiologic evidence of degenerative joint disease and synovial thickening. All eight patients had keratoconjunctivitis sicca, five of eight patients (62.5%) had Sjögren's syndrome, and five of eight patients (62.5%) developed rheumatoid cornea necrosis leading to corneal perforation.The implication exists that RA and OCP may be linked via an immunologically mediated mechanism and that patients with severe extraarticular symptoms associated with RA may be more likely to develop OCP. Prompt recognition of overlying symptoms may facilitate proper therapy for control of both diseases.

Purpose To describe the incidence and prevalence of glaucoma in a patient population with severe ocular surface disease (OSD). Methods A retrospective case series was compiled from all charts of patients in the Cincinnati Eye Institute/University of Cincinnati and University of Minnesota population with a diagnosis of severe OSD from 1991 to 2003. The incidence and prevalence of glaucoma in the overall patient population were identified, and stratified into disease subgroups. Results Of the 108 eyes evaluated in this study, 71 were diagnosed with glaucoma. The overall prevalence of glaucoma in patients with severe OSD is 65.7%, with a range from 42.9% to 88.4%. Analysis by subgroup shows the highest percentage of patients with concurrent glaucoma fall into the categories of aniridia and chemical injury, and the lowest was noted in those patients with autoimmune or iatrogenic OSD. Overall, the incidence of glaucoma was 20.4%, with a range of 13.6% to 60%. Conclusions Compared with previous studies, our results show a significantly higher prevalence of glaucoma in patients with severe OSD. This information warrants increased attention to treatment and management of OSD and concurrent glaucoma.

Purpose: To report a case of cicatrizing conjunctivitis as the initial manifestation of a bronchogenic squamous cell carcinoma. Methods: Case report of a 57-year-old patient with bilateral history of ocular pain, redness, and discharge, with no apparent predisposing factors. Examination revealed chemosis, pseudomembrane, and symblepharon formation, and multiple ulcerated pustular and vesicular periocular and perioral lesions literature review. Results: Skin biopsy result was compatible with bullous pemphigoid. Two months later he experienced respiratory symptoms, and a squamous cell carcinoma was diagnosed. Conclusions: This case demonstrates cicatrizing conjunctivitis as a possible paraneoplastic syndrome associated with squamous cell lung carcinoma.

Purpose To evaluate the use of the Boston keratoprosthesis type I implantation in patients with mucous membrane pemphigoid (MMP). Methods Retrospective review of 8 eyes of 8 patients with severe ocular surface disease and corneal blindness as a result of MMP who underwent Boston keratoprosthesis type I implantation at the Massachusetts Eye and Ear Infirmary from January 1, 2000, through December 31, 2009. The main outcome measures were best-corrected visual acuity, keratoprosthesis retention, and postoperative complications. Results The mean age of patients was 71.3 years (range, 55-94 years), and the mean duration of their disease preoperatively was 6.1 years (range, 1.7-11.4 years). Visual acuity after the surgery improved to 20/200 or better in 6 eyes (75%) and to 20/40 or better in 3 eyes (37.5%). Only 1 of 6 eyes (16.7%) was able to maintain visual acuity of 20/200 or better over a mean follow-up period of 3.2 years. Five of the 8 Boston keratoprosthesis type I devices (62.5%) extruded or had to be replaced during a mean follow-up time of 1.7 ± 1.7 years. Loss of vision to worse than 20/200 during the follow-up period occurred because of keratoprosthesis type I extrusion, end-stage glaucoma, and retinal or choroidal detachment. Conclusions The clinical outcomes of Boston keratoprosthesis type I implantation in MMP are guarded and, as judged from the literature, less favorable than those with the Boston keratoprosthesis type II for the same disease.

Cicatricial pemphigoid is an autoimmune systemic disease characterized by chronic conjunctival cicatrization. The natural history is one of variable degrees of progressive scarring of the ocular surface and visual loss. Previous reports have shown systemic chemotherapy to be efficacious in halting cicatrization in progressive cases. This observation makes it of critical importance to be able to detect progression of the disease as early as possible so as to allow for earlier institution of treatment. Our experience in previous studies has led us to believe that disease staging systems currently in use are relatively insensitive in detecting disease progression. We present a revised staging system that combines the strengths of the staging systems in use today and extends their sensitivity.

To describe a case of antiepiligrin cicatricial pemphigoid with unusual ocular manifestations and its remission after surgical removal of gastric carcinoma.We describe a 61-year-old Japanese man with antiepiligrin cicatricial pemphigoid.He presented with conjunctival injection and discharge preceded by a 6-month period of erosive lesions in the oral mucosa and the truncal skin. An advanced gastric carcinoma was found and his serum immunoprecipitated laminin-5. Despite topical treatment with betamethasone, ofloxacin, and artificial tear solutions, serious symblepharon along the Meibomian line developed with little shortening of the inferior conjunctival sac. Following radical gastrectomy, the ocular and cutaneous lesions turned completely quiet.The present case differed from past cases by lacking inferior conjunctival sac shortening and by showing erosive lesions solely at the mucocutaneous junctions. The ocular involvement in this case correlated very well with the severity of gastric carcinoma.

Osteo-odonto-keratoprosthesis (OOKP) is a keratoprosthesis technique in which the patient's own tooth root is used to support an optical cylinder. It was invented by Strampelli in 1963 and modified and established by Falcinelli about 10 years later. This method is particularly useful for restoring sight in end-stage Stevens-Johnson syndrome (SJS) and ocular cicatricial pemphigoid (OCP). We started performing OOKP surgery in June 2003 supported by Dr Christopher Liu of Sussex Eye Hospital, Brighton, UK. Till now, we have performed 4 OOKP surgeries for end-stage SJS and OCP. We modified the original method by using artificial buccal mucous membranes to the bone and buccal mucous membrane defects for better wound healing. Case 1 was a 49-year-old woman (SJS), and the corrected visual acuity was 0.5 in 4 years 8 months. Case 2 was a 68-year-old woman (SJS), and the corrected visual acuity was 0.04 in 3 years 10 months. Case 3 was a 63-year-old man (SJS), and the corrected visual acuity was 0.1 in 3 years 2 months. Case 4 was a 71-year-old woman (OCP), and the corrected visual acuity was 0.04 in 1 year 3 months. Despite some minor optical cylinder troubles such as MRSA colonization, tilting, and buccal mucous coverage, their visual acuities were stable without any serious complications. It was demonstrated that OOKP is useful for visual rehabilitation and durable with minimum eye care for severe ocular surface diseases.

Purpose: To describe a case of mucous membrane pemphigoid (MMP) with ocular manifestations in a patient subsequently diagnosed with B-cell chronic lymphocytic leukemia (CLL). Methods: Case report and literature review. Results: A 64-year-old man presented with redness and irritation of both eyes. The patient also had blistering lesions on the roof of his mouth, trunk, and buttocks as well as bilateral conjunctival ulceration and symblepharon formation. A complete blood count performed before initiating treatment of presumed mucous membrane pemphigoid revealed a markedly increased white blood cell count (83,920) with a predominance of lymphocytes. Hematologic evaluation including immunophenotyping flow cytometry resulted in the diagnosis of B-cell chronic lymphocytic leukemia. Cyclophosphamide treatment improved his MMP and decreased the white blood cell count. Conclusions: Mucous membrane pemphigoid has been reported in association with a variety ofnonhematologic malignancies. This case suggests that mucous membrane pemphigoid may also occur in B-cell CLL and supports the concept that autoantibodies play a role in the pathogenesis of this cicatricial conjunctival disease.

The purpose of this study was to analyze the distribution and types of collagen in the substantia propria of the conjunctiva of patients with ocular cicatricial pemphigoid (OCP). Biopsy specimens were collected from 10 patients with active OCP, five patients with active Behçet's disease, nine patients with atopic keratoconjunctivitis, five patients with chronic rosacea blepharoconjunctivitis, and six normal patients undergoing cataract surgery. Cryostat tissue sections were cut and stained using an indirect immunofluorescence technique, employing a panel of primary antibodies directed against seven collagen types. Differences between OCP, Behçet's, and normal conjunctiva were seen in the staining for collagen types III, IV, and VII. The intensity of staining for type III collagen was increased in the substantia propria of OCP conjunctiva as compared to the other groups. The basement membrane zone (BMZ) of OCP patients was typically disrupted and fragmented in appearance when stained for type IV collagen, a finding not seen in the non-OCP specimens. The BMZ staining pattern for type VII collagen in OCP conjunctiva was even more disrupted than that seen for type IV collagen, particularly on the posterior side, which was thickened and reduplicated with short fibers extending into the superficial stroma. The production of type III collagen by fibroblasts of the substantia propria is a common feature of diseases associated with subepithelial fibrosis. The damage to the epithelial BMZ and the subsequent attempt at repair with aberrant type IV and VII collagen production are unique to conjunctiva affected by OCP.

The records of 105 patients treated with three different chemotherapeutic agents for ocular cicatricial pemphigoid (OCP) were reviewed to compare long-term efficacies, side effects, and tolerance of different regimens. For the entire group, OCP progressed in 6% of eyes in 10% of patients (follow-up 35 months). More than half of the treatment failures occurred in patients intolerant of chemotherapy. Diaminodiphenylsulfone (DAP), as initial agent, failed to control disease in 2% of patients, compared with 8% after cyclophosphamide (CYC) and 9% after azathioprine (AZA) (p less than 0.05). Stratification of results revealed that DAP was the most effective initial agent for modestly active OCP, whereas CYC was the most effective initial choice for highly active cases. In patients treated with a single agent exclusively for 10 months or more, failure to control disease occurred in 4% of DAP, 4% of CYC, and 15% of AZA patients (p less than 0.01). Recommendations for a sequential approach to chemotherapy for OCP are presented.