2. Definition
• Crescent shaped destructive inflammation of
the juxta limbal corneal stroma
• associated with epithelial defect,
• stromal inflammatory cells
• and stromal degradation
3. • Usually conjunctiva, episclera and sclera are
also involved.
• Biopsy will show micro angiopathy.
• Antibodies to corneal epithelium is also found.
• In ANCA +ve vasculitides cell mediated
cytotoxicity is also found
4. DIFFERENCIAL DIAGNOSIS
• Peripheral degenerations like
• Arcus, White limbal girdle of Vogt
• Idiopathic furrow degeneration – thinning in
the area of arcus
• Marginal keratitis
5. D.D
• Post irradiation esp. Beta radiation
• Terrien’s marginal degeneration
• Bilateral, young males, superior, stromal
thinning, ectasia, lucid interval between the
lesion and the limbus.
• Yellow lipid border at the advancing edge.
8. Why limbus is involved?
• Limbal arcades
• Inflamed vessels draw inflammatory cells
which cause destruction
• Tight collagen bundles in the periphery
facilitates collection of IgM, C1 and other high
molecular weight molecules
• Immune complex deposits at the limbus and
corneal periphery
9. Why?
• Presence of Langerhans cells in the conj., and
corneal periphery
• Limbal macrophages (antigen presenting cells)
will be up regulated when there is inf., and
immune mediated events occur
• Channels from sub conj lymphatics enter the
periphery of the cornea- afferent arm
• Limbus is a reservoir of effector cells of
immune sys., cytokines
10. RHEUMATOID ARTHRITIS
• Autoimmune
• Typical joint deformities
• Ocular surface disorders more common
• Corneal melting can occur
• Following surgery PUK more common
11. Systemic Lupus Erythymatosis
• Dysfunction of immune regulation by T cells
and B cells
• Ocular surface disorder is more common
12. Wegener’s
• Necrotizing granulomatous vasculitis
• Bilateral PUK
• Lead on to ring ulcer
• Scleral inflammation is severe, may lead on to
necrosis
• Steroids not effective
13. Relapsing poly chondritis
• Cartilages involved
• Auditory impairment, tinnitus and vertigo
• Other collagen vascular disorders may co exist
• Ring ulcer with scleritis, bilateral – rare
• Lid edema, iritis, retinopathy, optic neuritis
• Cornea has glycosamino glycans- same as
sclera, aortic wall, cartilage and inner ear
14. Poly arteritis nodosa
• Non granumatous necrotizing vasculitis both
small and medium
• URI, drug abuse, hepatitis b and c
• Pseudo tumor, papilledema, papillitis, muscle
involvement
• Mooren’s like PUK
• Interferon alfa 2b. Steroids alone not effective,
cyclophosmamide to be added
15. MICRO POLYANGIITIS
• ANCA against myeloperoxidase
• Mooren’s like PUK, often bilateral with scleral
involvement
• Uveitis, ischemic optic neuropathy, muscle
palsies
16. MOOREN’S ULCER
• First described by Bowman in 1949
• Mooren published a paper later
• Type iii hypersensitivity ?
• Diagnosis by exclusion
• PUK may be the presenting feature of collagen
vascular diseases
17. • Primary – classic, idiopathic
• Secondary – trauma, surgical, chemical injury,
Herpes, syphilis, tuberculosis, Hepatitis C,
helminthiasis both ascariasis and ancylostoma
18. • Fulminating,
• centrally progressive,
• painful
• more common in males
• Under mining edge
• Sclera not usually involved
• Absence of any systemic disorder
22. Histology
• Necrosis of collagen tissue
• Chronic inflammatory cells
• Vascularisation of limbal margin
• Auto anti bodies to epithelium
• Polymorphs which are responsible for
collagenolysis
23. TREATMENT
• Steroids every hour
• Conjunctival excision- not just peritomy
• This helps by removing the causative factors
• Tissue adhesives – excludes neutrophils from
reaching the involved area
• Tetra cyclines to prevent collagenolysis
24. Immuno suppression
• Methotrexate 7.5 to 15 mg/week
• Azathioprine 2 mg/kg/day
• Cyclophosphamide 2 mg/kg/day
• The above three must be given for 6 months
and then tapered
• For Mooren’s topical cyclosporine can be used
• Before cataract surgery immune suppression
25. surgical
• Amniotic membrane graft
• Tectonic kp to prevent the immune complexes
from reaching the cornea
• Palliative, temporizing, inadequate to prevent
recurrences as immunologic process continues