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Thread: Cicatricial conjunctivitis capsule

  1. #1
    Join Date
    Oct 2017
    Riyadh, Saudi Arabia
    (Consultant Ophthalmologist at MALAZ MEDICAL GROUP)


    Default Cicatricial conjunctivitis capsule

    Cicatricial conjunctivitis capsule


    On request from a friend

    ✍️ conjunctival inflammation associated with scarring

    ✍️ rare

    ✍️ usually bilateral ( unless local causes)

    ✍️ sight-threatening group of disorders for which early diagnosis and appropriate treatment are essential.

    ✍️ loss of goblet cells, ocular surface failure (from chronic limbitis, limbal epithelial stem cell failure

    ✍️ progressive conjunctival scarring (hallmark of the disease).

    ✍️ onset may be insidious, delaying diagnosis

    ✍️ very easy to underestimate these conditions , so try to get expert help early .

    Cicatricial conjunctivitis capsule attachment.php?attachmentid=3459&d=1512898375

    Causes of conjunctival scarring

    Physical (heat and Ionizing radiation )

    Chemical (Alkali and Acid )


     Membranous conjunctivitis (Strept and adenovirus)
     Corynebacterium diphtheriae
     Chronic mucocutaneous candidiasis

    Oculocutaneous disorders

     Mucous membrane pemphigoid (MMP)
     Bullous pemphigoid (BP)
     linear IgA disease
     Dermatitis herpetiformis
     Pemphigus
     systemic lupus erythematosus
     epidermolysis bullosa aquista (eBA)
     ectodermal dysplasia
     SJS
     TEN
     lichen planus
     Chronic atopic keratoconjunctivitis

    Other associated systemic disorders

     rosacea
     sjögren’s syndrome
     Inflammatory bowel disease (IBD)
     GVHD
     Immune complex diseases
     Paraneoplastic syndrome
     sarcoid
     Porphyria

    Drug-induced cicatrizing conjunctivitis

     antiglaucoma medication( pilocarpine and timolol)
     practolol
     penicillamine
     topical (propine , idoxuridine, gentamicin and guanethidine).


     OSSN (squamous cell or sebaceous cell carcinoma)
     lymphoma

    Cicatricial conjunctivitis capsule attachment.php?s=027180f8df4a090b2f250c113485f37e&attachmentid=3460&d=1512898403

    Ocular Mucous Membrane Pemphigoid (OCMMP)

    ✍️ chronic inflammatory subepithelial blistering disease of the mucous membranes.

    ✍️ usually occurs >60y of age but may occur in adolescents, in whom the disease is more severe

    ✍️ it is slightly more common in female .

    ✍️ there is an association with other autoimmune disease (RA and pernicious anaemia).

    ✍️ type II hypersensitivity reaction, with linear deposition of immunoglobulin and complement at the BM zone of mucosal surfaces.

    ✍️ Although the target antigens are known (the antibodies are specific to components of the BM and hemidesmesomes), the triggering agents for the disease are not clear.

    ✍️ oral mucosa and conjunctiva are most commonly affected, although skin and other mucous membranes may be involved.

    ✍️ Involvement of the trachea or oesophagus is potentially life-threatening.

    ✍️ diagnosis of MMP requires direct immunopathological confirmation as well as typical clinical features,

    ✍️ OCMMP, diagnosis is primarily clinical, with immunopathology providing supporting evidence only.

    ✍️ Clinical features

    • Irritation.
    • Acute and chronic papillary conjunctivitis
    • subconjunctival bulla
    • ulceration
    • progressive cicatrization
    ☝️loss of plica semilunaris
    ☝️loss of fornices
    ☝️ formation of symblepharon
    ☝️ ankyloblepharon
    ☝️ trichiasis
    ☝️ cicatricial entropion)
    ☝️ dry eye
    ☝️ microbial keratitis
    ☝️ corneal neovascularization
    ☝️ ulcerative keratitis and perforation
    ☝️Limbal Stem Cell failure and keratinization.

     NB exclude infection as a cause of inflammation before jumping and attributing inflammation to the disease process

    ✍️ risk factors
    ☝️ poor ocular surface
    ☝️ lid trauma
    ☝️ immunosuppression.

    ✍️ Treatment

    ☝️ early diagnosis and treatment improves outcome.
    ☝️ refer early for specialist help.
    ☝️ Adnexa
    • ensure lids and lashes are not a cause of inflammation.
    • early conjunctiva-sparing lid surgery is vital.

    ☝️Maximize tear film stability
    • punctal occlusion
    • tear substitutes
    • treat MGD and blepharitis.

    ☝️Exclude infection.

    ☝️once all 2ry causes of inflammation (dry eye, lash or lid trauma or infection) are treated , any residual inflammation is disease-related to OCMMP

    ☝️ Immunomodulation:

    •topical corticosteroids(with caution as it may mask disease.
    • Doxycycline 50–100mg/d for 3mo (MMP inhibitor, pregnancy, lactating and children are contraindicated).
    • Disease-modifying agents:
    step-up strategies
     dapsone if mild
     mycophenolate, methotrexate, or azathioprine if moderate
     cyclophosphamide if severe
     combination treatments, according to drug action, may be required.
     All need monitoring for response and side effects
     systemic immunosuppression is generally required for >1y.

     For persistent/resistant cases IV immunoglobulin, anti-CD20 or anti-tumour necrosis factor (anti-tNF) therapies.


     Clinically quiescent eyes may have occult inflammation, resulting in disease progression.
     Monitor scarring with photography and measurements (fornix depth measurements ).
     Treat complications
     entropion
     trichiasis
     fornix obliteration (may require
    oral mucosal grafting to reconstruct fornix)
     persistent epithelial defects (exclude infection first)
     limbal stem cell failure
     corneal exposure (botulinum toxin is of limited use, due to mechanical restriction, but can be effective in a few)
     corneal perforations.
     end-stage disease may require osteo-odonto-keratoprosthesis.

    Other causes of cicatrizing conjunctivitis

    ✍️ Erythema multiforme
    ✍️ SJS
    ✍️ TEN (Lyell disease)

     these are acute vasculitides of the mucous membranes and skin
     associated with drug hypersensitivity (sulfonamides, anticonvulsants, allopurinol) or infections (mycoplasma, HSV).
     triggers cause T-cell activation and immunological cascades

    Clinical picture almost the same

    ✍️ TEN is systemically the most severe
    ✍️ characterized histologically by keratinocyte apoptosis and clinically by >30% body involvement. ✍️ Mortality rates are 30–40%.
    ✍️ those who survive, ocular disease may be persistent, whilst the systemic disease subsides.

    ✍️ Clinical features
    ☝️ Acute fever and malaise
    ☝️ skin rash (target lesions or bullae)
    ☝️ haemorrhagic inflammation of mucous membranes
    ☝️ sloughing of epidermal surfaces (Nikolsky’s sign).
    ☝️ Papillary or pseudomembranous conjunctivitis cicatrization
    ☝️ ocular disease progression and ocular surface failure, can occur years after the acute illness.

    ✍️ Treatment

    ☝️ expert multidisciplinary care is required
    ☝️ treated in regional burns unit where possible.
    ☝️ Topical: tear substitutes, corticosteroids, and antibiotics (PF).
    ☝️ Systemic immunosuppression: controversy surrounds the use of corticosteroids (systemic), as they have a role for ocular disease but may have a negative effect on general disease and prognosis. ☝️ there is a possible role for IV immunoglobulin and IV ciclosporin.
    ☝️ Consider surgical division of adhesions and careful removal of membranes
    ☝️ symblepharon rings are controversial as may become locked in conjunctival fibrosis if forgotten)
    ☝️ gas-permeable scleral Cl
    ☝️ conformers (vault lids away from bulbar conjunctiva)
    ☝️ AMG to protect ocular surfaces and lids (john’s procedure), or use amnion mounted on a scleral skirt (ProKera) or an amnion-wrapped conformer, all combined with or without subtarsal triamcinolone 20mg each tarsus
    ☝️ Management of chronic sequelae are as for OCMMP or scleritis


    ✍️ Graft Versus Host Disease
    ✍️ occurs in some allogeneic bone marrow transplant patients
    ✍️ the donor’s leucocytes attack the immunosuppressed recipient.
    ✍️ commonly affects the ocular surface, although rarely posterior segment features (posterior scleritis, choroidal thickening) during the acute stage.
    ✍️ In the acute response, there is TEN-like response, which may include pseudomembranous conjunctivitis.
    • stage I hyperaemia
    • stage II hyperaemia with serosanguineous chemosis
    • stage III pseudomembranous conjunctivitis
    • stage IV pseudomembranous conjunctivitis with corneal epithelial sloughing.

    ✍️ In chronic GVHD, scleroderma-like changes of the skin and sjögren’s-like changes of the glands to cause dry eye and cicatricial changes of the conjunctiva.

    Attached Images  
    Last edited by Reda Gomah El Garia; 12-10-2017 at 09:34 AM.

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