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Localized EBS
most common form EBS
onset : in childhood or later
clinically :
- theck-walled blisters on the
hands & feet
- occur ; after excessive exercise,
manual
work
Junctional EB
- Blister formation within lamina lucida
- Clinical feature depending on the type of
genetic lesion & environmental factors
JEB Gravis :
- often do not survive infancy 40%
mortality rate during first year-old
- generalized blistering at birth
- distinctive & severe periorificial
granulation
- loss of nail
- sometimes completely denuded oozing
painfull erosion
JEB Mitis :
- moderat or severe JEB at birth clinically
improve with the age
DX : - clinical appearance & history
- histopathology : immunochemical mapping
Tx : syst : for complication
wound management
nutrional support
infection control
Pemphigus Vulgaris
Pemphigu
Skin
lesions :
Skin lesions:
- localized/generalized
- Erythematous , papular or urticarialtype lesions may precede bullae
formations by months.
- Bullae : large, tense, firm-topped,oval &
round
arise in normal / erythematous skin &
contain serious or hemorrhagic fluid.
a group arciform/serpigenous pattern
ruptur less easily than in pemphigus
Sites of predilections :
- axillae, thighs, groins, abdomen,
medial
forearms, lower legs
- mucous membranes mouth
Dx : clinical appearance & histopathology
; subepidermal bullae, neurotrophil at
dermal-epidermal junction
DD : other bullous diseases
Tx: -Systemic:
- prednisone started 50-100mg/d
clear /combined with
Azathioprine
150mg/d
- Dapsone 100-150mg/dmilder
cases
-Topical steroid very mild cases
Skin lesions :
- erythematous papules/wheal-like
plaques, tiny firm-topped vesicles,
sometimes hemorrhagic bullae
- a group lesions herpetiformis
- distribution strikingly symetric
- scratching excoriations & crusts
Predilection : extensor elbows, knees
typical, buttock, scapular & sacral area
Histopathology:
mycroabcesses (polymorphonuclear
cells &
eosinophils) at tips of the
dermal papillae
DX : clinical appearance that
accompanied by
severe pruritus
DD: -allergic contact dermatitis
- atopic dermatitis
- papular urticaria
- bullous pemphigoid
- pemphigoid gestationis
Tx : systemic :
- Dapsone 100-150mg/d
reduction
50-25mg
50mg (2x/w)
- Sulfapyridine:1-1,5gr/d dapson
contraindicated
Diet : gluten-free
Pemphigoid Gestationis
Def : pruritic & polymorphic inflamatory
bullous dermatosis of pregnancy and
post partum period, mainly on
abdomen
usualy begins :
- 4-7month of pregnancy,
- immediate post partum periode
Tx : - prednison 20-40mg/d
- antihistamine & topical steroid mild