Professional Documents
Culture Documents
KRONIK
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Chronic bullous diseases
1. Pemphigus
a. Pemphigus vulgaris
b. Pemphigus eritematous
c. Pemphigus foliaseus
d. Pemphigus vegetans
2. Bullous pemphigoid
3. Dermatitis Herpetiformis/ Duhring diseases
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o Desmosomes are molecular complexes of cell
adhesion proteins and linking proteins that
attach the cell surface adhesion proteins to
intracellular keratin cytoskeletal filaments
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Pemphigus vulgaris
Pemphigus vulgaris is characterized by IgG
autoantibodies against desmoglein 3 (Dsg 3),
although about 50% of PV patients also have
Dsg1 autoantibodies.
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• Symptoms
Multiple flaccid blisters that rupture
easily, leaving raw painful areas.
Ulcers in the mouth.
Usually affects the middle-aged and
elderly.
Nikolsky sign
Tanda Asboe-Hansen (bulla-spread
phenomenon.
40-60 age
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Diagnosis
• appearance and distribution of the skin
lesions
• histopathology
• direct immunofluorescence
• ELISA
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Treatment
• The most common treatment is the
administration of oral steroids, especially
prednisone, often in high doses. The side
effects of corticosteroids may require the
use of so-called steroid-sparing or
adjuvant drugs.
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Bullous pemphigoid
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Sign & symptoms
• 70 years of age and older
• Earliest lesions → urticarial (like hives)
• Tense bullae → inner thighs and upper arms,
also trunk and extremities
• Any part of the skin surface can be involved
• Oral lesions are present in a minority of cases
• Nikolsky's sign is negative unlike pemphigus
vulgaris where it is positive
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Bullous pemphigoid → IgG and activated T
lymphocytes (white blood cells) attack
components of the basement membrane,
particularly a protein BP antigen BP180, or less
frequently BP230. These proteins are within the
NC16A domain of collagen XVII. BP180 is also
called Type XVII collagen. These are
associated with the hemidesmosomes
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Diagnosis
• appearance and distribution of the skin
• lesions
• histopathology
• direct immunofluorescence
• ELISA
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• DIF tests in patients with bullous pemphigoid
usually demonstrate immunoglobulin G (IgG)
and complement C3 deposition in a linear
band at the dermal-epidermal junction, with
IgG in salt-split skin found on the blister roof
(epidermal side of split skin).
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Treatment
Treatment usually includes corticosteroids,
such as prednisone, and other drugs that
suppress the immune system. Bullous
pemphigoid can be life-threatening,
especially for older people who are already
in poor health.
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