Pemphigus and pemphigoid

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• Pemphigus and pemphigoid

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(pemphigus)

• definitionautoimmune skin and mucous membrane
  vesiculobullous disease is characterized with
• acantholysis,bullae formation in the
  intraepidermal areas caused by
• intercellular antibodies deposited in stratum
  spinosum

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classificationfour types two species

• vulgaris type pemphigus vulgaris
• (bullae at underlayer
• of acanthocytes)
• pemphigus
  vegetans
• foliaceus type pemphigus foliaceus
• (bullae at superficial
• layer of acanthocytes)
• 
  pemphigus erythematosus

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pathogenesy

• Evidence of autoimmune disease
• Ig deposited between acanthocytes of skin lesion
• Pemphigus antibodies are present in serum
• antibody titer related with
  pathogenetic condition
• Plasmapheresis removing pemphigus antibodies is
  useful in a short term
• pemphegus antibodies transfer fetus across
  placenta
• High titer pemphigus antibodies repeatedly
  intradermal injection induced acantholysis

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Desmoglein 3
desmoglein 3
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Clinical features

• Incidence of pemphigus per year0.53.2/100,000
• 1. Pemphigus vulgaris
• Usually occurs in middle-age(age of 60)
• Blister and erosion usually appear on oral mucosa
• thin-walled,flaccid bullae or enlarged erosion
  suface
• Nikolsky sign positive
• Lesions appear in the scalp,face,chest and back

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2. pemphigus vegetans

• Hallopeau
• Nenmann
• The lesions limited in axillae?groin ?under
  breast?perineum, etc.

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3. pemphigus foliaceus

• Lesions are at upper layer of acanthocytes
• Thin-walled bullae or porous thick crusts
• Lesions aggravate after exposed to sunlight

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pemphigus erythematosus

• lesions appear most commonly in the
  scalp?face?superior part of trunk
• Erythema and flaccid bulla
• Nikolsky sign ()

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Immunologic test

• direct immunofluorecence (DIF)
• in acantholytic areas
• IgG? IgM?IgAor C3 depostion

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Indirect immunofluorecence

• (IIF)
• Circulating intercellular antibodies can be
  demonstrated in 8090 of patients serum with
  pemphigus vulgaris
• Mainly IgG
• Antibody titer often parallel disease activity

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histopathology

• pemphigus vulgaris
• Blster intraepidermal
• Acantholysis in bullae
• (pemphigus cell)

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Diagnosis and antidiastole

• Pemphigus vulgaris(1)
• Usually occurs in the elderly(around age of 60)
• Blister and erosion usually appear on oral mucosa
• thin-walled,flaccid bullae or enlarged erosion
  suface
• Nikolsky sign positive
• Lesions appear in the scalp,face,chest and back

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Diagnosis and antidiastole

• Pemphigus vulgaris(2)
• DIF shows Ig deposited intercellular
• IIF shows pemphigus antibodies
• Pathology shows blister intraepidermal,acantholysi
  s

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treatment

• 1. General treatment
• Surpportive therapy?vitamin and protein
  supplement
• Transfusion and eleltrolyte balance
• Attending raw surfaces,prevent infection
• Caution the complication

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• 2. corticosteroid
• choice drug,early?full dose?maintenance
• 3. Immunosuppresant
• azathioprine or cytoxan
• 4.dapsone(D.D.S)
• 5.wilfordii preparation

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• 6. plasmaphoresis
• 7. Large dose IVIg stosstherapy
• 0.4g / kg / d 35d

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pemphigoid

• definition
• An autoimmunity dermatosis mainly occurs in
  aged people ,characterized with blister formation
  intraepidermal

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classification

• Bullous pemphigoid ( BP)
• Localized pemphigoid
• ???????
• ????????
• Pleomorphous pemphigoid
• Cicatricial pemphigoid

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Pemphegoid clinical features(BP)

• usually occurs in the elderly(over age of 70)
• thic-walled tense bullae or blister
• Maily apear in axillae?groin
• Histopathology shows bullae subepidermal
• DIF shows C3 and/or IgG linear deposited in the
  basement of the membrane zone

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treatment

• 1. General treatment
• High protein ingest
• Prevent skin secondary infection
• Eliminate malignant tumor

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• 2. corticosteroid
• choice drug,early?full dose?maintenance
• 3. Immunosuppresant
• azathioprine
• 4.dapsone(D.D.S)
• 5.wilfordii preparation
• 6. Tetracycline plus nicotinamide
• 7. plasmaphoresis
• 8. Large dose IVIg stosstherapy