Vesicullobullous.txt

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nehashah88
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89151
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Vesicullobullous.txt
Updated:
2011-06-04 18:12:41
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vesiculo bullous lesions
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vesiculo-bullous lesions
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  1. V-B lesions can be classified as:
    • Primary e.g. Pemphigus
    • Secondary e.g. physical, chemical burn
    • Congenital
  2. Most primary causes of V-B lesions are?
    Immunobullous disorders
  3. Give examples immunobullous disorders PPLED?
    • Pemphigus
    • Pemphigoid
    • Linear IgA
    • Epidermolysis Bullosa
    • Dermatitis Herpetiformis
  4. Give eg.s of congenital causes:
    Epidermolysis bullosa
  5. They result from?
    Loss of attachment between cells (intraepi) or between cells and CT (sub-epi)
  6. What are most V-B disorders?
    • All sub-epi
    • EXCEPT pemphiGUS Vulgaris and viral infections
    • EM can have both
  7. Of the intra-epi lesions, name the two mechanisms?
    • Pemphigus - acantholytic (breaks down the attachment apparatus desmosomes)
    • Viral - non acantholytic (cell death)
  8. Pemphigus types:
    • Most common Vulgaris (70%)
    • Vegetans
    • Paraneoplastic
  9. Who gets Pemphigus?
    • Middle age
    • Jewish/Italian/Mediterranean
    • Female>male
  10. Clinical fts Pemphigus:
    • SKIN - limbs, trunk, genitalia, lip and nose crusting
    • MUCOSA - Blisters, become erosions, desquamative gingivitis, +Nikolsky�s sign
    • Other mucosa, e.g. conjunctiva
  11. Histopath of Pemphigus:
    • Inta-epi bulla produced by acantholysis
    • Tzank cells in vesicle fluid
    • Rupture of vesicle - ulcer with inflamm infiltrate in floor
  12. Investigations
    • DIF - IgG to intra-cellular epi
    • IIF - (serum)- increase titre of circulating antibodies Desmoglein 3 (and some pts Desmoglein 1)
  13. How can you monitor the condit?
    Increase titre indicates increased severity
  14. Treatment of pemphigus?
    • Steroids eg. 80-100g/day
    • and Azathioprine - allow steroid dose to be lower
    • Screen pts before giving Aza, they might have an enzyme breaking it down
    • Consult dermatologist, opthamologist
  15. Paraneoplastic pemphigus is assoc with?
    • partic lymphomas and leukaemia
    • Usually resolves if cancer resolves
  16. Pemphigoid, 2 major clinical types?
    • Bullous Pemphigoid - SKIN
    • MMP - Mucosal (skin rare)
  17. Who gets MMP?
    Similar to Pemphigus, Middle aged, female
  18. Name some of the mucosal surfaces affected:
    • OM (>90%)
    • Conjunctiva (>90%)
    • Nasopharynx 35%
    • Larynx, oesophagus
  19. What do they get with MMP?
    • Bullae/erosions (if ruptured)
    • Can be blood filled
    • Erosions are longer lasting, persistent, slow to heal, fibrinous base
    • Dequamative ging common
    • May have +Nikolsky
  20. Histo of MMP?
    • Sub epi bullae
    • The epi forms roof of bullae
    • CT is floor
    • CT infiltrated with inflamm
  21. Investigations for MMP
    • DIF - binding of anti C3/ IgG/M to BM
    • IIF - generally low levels of Autoantibodies, can be -ve even!
  22. Treatment of MMP?
    • Mild - topical steroids
    • Mod-severe - dapsone, prednis + azathioprine
  23. Is MMP fatal?
    • No, but has a chronic course, can lead to blindness, therefore Tx as early as poss.
    • Opthalmic assess
  24. Complications of MMP:
    • Blindness from conjuntival scarring
    • Laryngeal scarring - stenosis
    • Oesophageal scarring - stricture
  25. MMP diff dx?
    • Pemphigus
    • Linear IgA
    • Angina bullosa haemorragica
    • Erosive LP
  26. Linear IgA name 4 fts?
    • Sub epi
    • Very similar to MMP
    • Oral and occ
    • DIF linear deposition of IgA at BMZ
  27. Dermatitis Herpetiformis 4 fts?
    • Rare, usually assoc with Coeliac
    • Skin - pruritic rashes at elbow, knees
    • OM in only 10% pts (bullae, desquamitive ging�)
  28. What is Erythema multiforme?
    Acute, often recurrent, HYPERSENSITIVITY REACTION.
  29. Possible causes of EM?
    • Idiopathic
    • Drug reaction eg. Anticonvulsants, barbituates, sulphonamides, antibiotics
    • Infection eg. H. simplex
    • Other eg. Preg, malig
  30. Who gets it?
    • Younger people 20-40yo
    • 20% in children
  31. Describe the spectrum of clinical fts of EM:
    • Minor - affects only 1 site, not debilitating
    • Major - widespread, life threatening, Steven-Johnson syndrome
  32. What is EM often characterised by?
    • RAPID ONSET
    • Serosanguinous exudates of the lips
    • Oral ulceration
    • Other mucosa
    • Target lesions on skin
  33. Rashes in EM can vary, describe:
    Patchy erythema to target lesions and bullae
  34. What makes you think viral infection as a differential?
    Prodromal symps may be experienced
  35. Steven-Johnson syndrome involves which sites?
    Skin + 2 or more mucosal lesions
  36. Oral lesions of SJS?
    • Widespread irreg fibrin covered erosions
    • Diffuse and widespread macules that progress to blisters and ulceration
    • Lips - swollen, cracked, bleeding, crusting, drooling
    • Recurrence. Lasts 10-20days
  37. Skin lesions of SJS?
    • Macules/Papules - pruritic or erythematous
    • Large target lesions - centre is a necrotic ulcer
    • If very severe, large skin bullae - Toxic Epidermal necrolysis
  38. Other mucosa in SJS?
    • Eyes - lacrimation, redness, bloodshot, conjunctivitis, adhesion of eyelid to eyeball
    • Genital lesions
  39. Dx of EM?
    • Clinical
    • Serology (cause?)
    • Biopsy - Variable histopatho - sub and intra epi
  40. EM Tx?
    • Admit
    • Fluids, Nutrients if anorexia
    • PEG, nasogastric
    • Tx cause
    • Corticosteroids

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