Ocular cicatricial pemphigoid

Hingorani, Melanie; Lightman, Susan

doi:10.1097/01.all.0000244799.33734.d4

Cited by 23 publications

(10 citation statements)

References 39 publications

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“…The destruction of Vogt Palisades and limbal stem cells deficiency could be similarly found in other chronic inflammation on ocular surface such as Stevens-Johnson syndrome and cicatricial ocular pemphigoid. 19,20 However, the relationship between the duration and severity of VKC and the degree of Vogt Palisades destruction requires further investigation with a large number of subjects.…”

Section: Discussionmentioning

confidence: 99%

In vivo laser scanning confocal microscopy of vernal keratoconjunctivitis

Hong

Zhu

et al. 2010

Clinical &Amp; Experimental Ophthalmology

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Section: Discussionmentioning

confidence: 99%

In vivo laser scanning confocal microscopy of vernal keratoconjunctivitis

Hong

Zhu

et al. 2010

Clinical &Amp; Experimental Ophthalmology

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“…A typical positive result will reveal in the epithelial basement membrane a linear deposition of different immunoglobulins (IgG, IgA, IgM) and complement 3 proteins. Still, 20-40% of patients have a negative biopsy result that does not rule out the diagnosis ( 14 , 15 ). Radioimmunoassay and immunoblot techniques can identify the circulating autoantibodies in active disease while classic indirect immunofluorescence is not helpful.…”

Section: Diagnosis and Classificationmentioning

confidence: 99%

Ocular cicatricial pemphigoid (Review)

et al. 2020

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Ocular cicatricial pemphigoid is a particular form of mucous membrane pemphigoid and it is characterized by a chronic bilateral conjunctivitis with relapsing-remitting periods. Without therapy 75% of the cases develop visual loss due to major ocular complications (e.g. severe dry-eye syndrome, corneal erosions, corneal keratinization, entropion, symblepharon). Pathogenesis remains uncertain and probably linked to an autoimmune type II hypersensitivity response in patients with a genetic predisposition and exposure to different environmental triggers. With a worldwide distribution, no racial predilection and an estimated incidence that largely varies from 1/10,000-1/60,000, ocular cicatricial pemphigoid predominantly affects women aged ~60 years. Conjunctival biopsy with direct immunofluorescence is the gold standard in diagnosis confirmation, but up to 40% of the patients have a negative biopsy result that does not rule out the diagnosis. The skin and many other mucous membranes (e.g. oral, trachea, esophagus, pharynx, larynx, urethra, vagina and anus) may be involved. The disease grading relies on Foster staging system (based on clinical signs) and Mondino and Brown system (based on the inferior fornix depth loss). The differential diagnosis includes atopy, allergies, trauma, chemical burns, radiation, neoplasia, infectious, inflammatory and autoimmune etiologies. The main goals of the treatment are to stop disease progression, to relieve symptoms and to prevent complications. With long-term systemic therapy 90% of the cases can be efficiently controlled. While Dapsone is the first-line treatment in mild to moderate disease in patients without G6PD deficiency, more severe cases require immunosuppressant therapy with azathioprine, mycophenolate mofetil, methotrexate or cyclosporine. Cyclophosphamide, biologics (etanercept or rituximab) and intravenous immunoglobulin therapy are usually reserved for recalcitrant disease and unsatisfactory results to conventional therapy. Dry eye syndrome requires constant lubricating medication and topical steroids, cyclosporine-A and tacrolimus. Surgery should be planed only in quiescent phase as minor conjunctival trauma can significantly worsen the disease.

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“…There have been some reported cases of CP triggered by human immunodeficiency virus, diphtheria vaccination, and some medications such as methyldopa, clonidine, and D-penicillamine [110,111]. In addition, other autoimmune diseases may occur more frequently in patients with CP [112]. Moreover, some bullous diseases may indicate the presence of an underlying malignancy.…”

Section: Etiopathogenesismentioning

confidence: 99%

“…These patients are clinically indistinguishable from other autoimmune bullous diseases. Specialized immunopathologic studies and/or IEM may be required for diagnosis [112,125]. It also has been reported that ocular CP developed after Steven Johnson syndrome and Lyell syndrome in some cases.…”

Section: Differential Diagnosismentioning

confidence: 99%

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

Ayvaz¹,

Gönül²,

Atay³

2018

Autoimmune Bullous Diseases

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Bullous systemic lupus erythematosus is a rare distinctive subepidermal bullous disease seen in patients with systemic lupus erythematosus (SLE). It has characteristical clinic, pathologic, and immunologic findings including antibodies to type VII collagen, laminin 332, laminin 331, and bullous pemphigoid antigen 230. Clinical presentation combined with histopathology, immunological testing, and concomitant diagnosis of SLE according to the criteria of American College of Rheumatology, are required to distinguish bullous SLE from these bullous diseases. In patients with bullous SLE, SLE disease progression and complications may be worse. Cicatricial pemphigoid is a chronic subepidermal blistering disease which is characterized by erosive lesions of mucous membranes and skin. Pathogenesis of cicatricial pemphigoid is characterized by linear deposition of Immunoglobulin G, A, or complement 3 along the epithelial basement membrane zone. The main target antigens are bullous pemphigoid antigens 180-230, laminin 331-332, type VII collagen, and β-4 integrin subunit. Cicatricial pemphigoid may lead to serious complications such as blindness and airway obstruction. Herein, clinical, histological, immunopathological features, the diagnosis and treatment of bullous SLE and cicatricial pemphigoid diseases are mentioned to raise awareness among the dermatologists about this important but rare heterogeneous bullous disease.

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Ocular cicatricial pemphigoid

Abstract: OCP remains a difficult disease to manage well and the progress being made in understanding disease mechanisms is whence true disease-modifying, safe therapy is likely to emerge in the future.

Cited by 23 publications

References 39 publications

In vivo laser scanning confocal microscopy of vernal keratoconjunctivitis

In vivo laser scanning confocal microscopy of vernal keratoconjunctivitis

Ocular cicatricial pemphigoid (Review)

Bullous Systemic Lupus Erythematosus and Cicatricial Pemphigoid

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