???(Pemphigus)??

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• ?????

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???(Pemphigus)??

• ??????
• ?????
• Immunoglobulins and complements are found bound
  to the intercellular substance in the
  perilesional epidermis

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???(Pemphigus)???

• World-wide distribution
• MF , middle age
• ?? D-penicillamine, etc.

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???(Pemphigus)????

• ????? ?????Flaccid vesicles and bullae
• Nikolskys sign ??
• ???? gt 50 of patients, as the initial
  lesions--generalized bullous phase in most
  patients occurs some 5 or more months after the
  onset of oral oral lesions.
• ??? face, trunk, pressure points, groin ,
  axillae
• ???? without scarring but transient
  hyperpigmentation common.

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???(Pemphigus)

• ?? Suprabasal separation with acantholysis
• ??????
• --??(D I F) IgG C3 at intercellular space
  --??(I I F) -Intercellular antibody 80-90
  -Titers often parallel disease activity

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???(Pemphigus)??

• ????? high dose
• Immunosuppressive therapy -- Imuran, diminish
  the need for corticoseroids

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????(Bulous Pemphigoid)??

• ??????
• -starts with urticaria-like and pruritic
  erythematous lesions, later, large dense blisters
  in both erythematous and normal skin.
• ????? intact epidermis forms the roof.

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????(Bulous Pemphigoid)

• Age 65 75 y/o
• Sex MF
• Bullous pemphigoid antigens are hemidesmosomal
  components, located below the basal epithelial
  cells.

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????(Bulous Pemphigoid)????

• ????Commonly starts with non-specific rashes on
  the limbs
• -???? 1-3 weeks before blisters occur
• -??? several months.
• -General eruption of pemphigoid follows the
  prodromal phase and most of the body may be
  affected within a week.
• -arise on erythematous or normal skin and may be
  associated with subcutaneous edema.
• ????? flexural aspects of limbs and abdomen.
• ???? ??

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????(Bulous Pemphigoid)

• ?? subepidermal blister, mainly eosinophils
• ??(D I F) IgG and C3 along the basement membrane
  zone.
• ??(I I F) circulating IgG attack basement
  membrane zone

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????(Bulous Pemphigoid)??

• ???? topical or intralesional steroids
• Systemic corticosteroid
• Tetracycline, Erythromycin, Niacinamide
• Azathioprine,dapsone

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?????(erythema multiforme EM)

• ??
• EM minor,EM major or SJS, and TEN.
• ???
• ?? herpes simplex or mycoplasma, less severe
• ?? 10. severe

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Stevens-Johnson syndrome(SJS)

• ?? young adult males
• ?? high fever, asthenia, muscular pains,
  diarrhea, vomiting, arthralgias, and pharyngitis
  precede mucosal involvement by several days.
• ???? 2 or more mucosal sites

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Toxic epidermal necrolysis(TEN)

• ??skin tenderness that resembles a severe
  sunburn.
• ????20 to 100 TBSA.
• Flaccid bullae may form. Nikolsky sign is
  positive. Two or more mucosal sites are involved
  in 85-95 of patients with TEN.
• ??? 30.

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???????

• antibiotics (sulfonamides, trimethoprim-sulfametho
  xazole, penicillins, cephalosporins,
  chloramphenicol, clindamycin, griseofulvin,
  rifampin, streptomycin, tetracycline)
• nonsteroidal anti-inflammatory agents (ibuprofen,
  acetylsalicylic acid, ketotifen, naproxen,
  piroxicam, sulindac)
• antihypertensives,
• anticonvulsants (phenobarbital, carbamazepine,
  phenytoin)
• allopurinol.

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????

• ?????? interface dermatitis with necrotic
  keratinocytes
• TEN shows massive and confluent necrosis of the
  basal cells

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??

• ?????
• ??????
• ??????? may abort the process, but it will not
  restore epidermis that is already necrotic.
• Burn unit care

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??

• EM most often has a good prognosis
• SJS and TEN can be lethal depending on the extent
  of skin involvement and the age of the patient.