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VESICULOBULLOUSDISORDERS
VESICLE : fluid filled blister < 0.5 cm in size.
BULLA : fluid filled blister > 0.5 cm
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CONGENITAL INHERITED DISORDERS
Epidermolysis bullosaHailey-2 disease
Relapsing linear acantholytic dermatosis
IMMUNOLOGICAL DISORDERS
Intraepidermal -Pemphigus
Subepidermal - Bullous pemphigoidLinear IgA disease
EBA (epidermolysis bullosa acquista)
Bullous SLE
Dermatitis herpetiformis
CLASSIFICATION
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PEMPHIGUS
Pemphix blister/bubble
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Variants of pemphigus
P.vulgaris
P.vegetans
P.foliaceusP.erythematosus
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Pemphigus vulgaris
Pathogenesis
Autoantibodies- Desmoglein
( present over desmosomes)
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Pathology
- Suprabasal split
- Row of tombstones:
Basal cell attached to B.M
-but separate from one another-Acantholytic cells in blister cavity
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Clinical features
-Oral lesions in all patients
-Other mucosal sites may be involved
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Predilection forScalp, face, axillae, groins,
pressure points
Lesions
Flaccid blisters filled with
clear fluid later fluid becomes
turbid/ rupture to form erosions
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Nikolskys Sign
Positive(firm sliding pressureseparates normal looking
epidermis from dermis)Bulla spread Sign : Positive
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Conjunctival involvement
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Mucosal involvement
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Mucosal involvement
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Mucosal involvement
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Mucosal involvement
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Cutaneous lesions
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Cutaneous lesions
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Cutaneous lesions
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Pemphigus foliaceous
Blistering just below corneum
Blistering may not be obvious
Antigen - Desmoglein 1
Upper part of body involved
Mucosae - spared
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Pemphigus erythematosus
Erythematous, scaly lesions
Nose / cheeks
(butterfly distribution)
Lesions over trunk
similar to P.foliaceous
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Pemphigus erythematosus
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P. vegetans
Vegetating erosions in flexures
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P. vegetans
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Paraneoplastic pemphigus
Underlying malignancy
Drug Induced pemphigus
Pencillamine, Penicillin, Captopril, Enalapril,
Cephalosporins & Rifampicin
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Treatment of pemphigus
Corticosteroids 1.0 1.5 mg/kg/day
(Pulse therapy,oral regime)
Azathioprime 2.5mg/kg/day
Cyclophosphamide 1-3 mg/kg
Cyclosporin 5 mg /kg/day
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Bullous pemphigoid
-Blistering disease of elderly
Tense , large bullae
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Etiology and Pathogenesis-Not well defined
Drugs-
(Pencillamine,captopril,
antipsychotics,
aldosterone antagonists)
-HLADQ7
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Histopathology
Subepidermal blister
Dermal inflam. infilt.
Eosinophils,
neutrophils,
lymphpcytes
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Clinical features
Urticaria like /or eczematous rash
Bullous lesions- after wks to monthsTense, dome shaped,
upto dia. of 7 cm
Flexural aspects of limbs/abdomen
Remain intact for several days
General condition Good.
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Bullous pemphigoid
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Bullous pemphigoid
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Bullous pemphigoid
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Bullous pemphigoid
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Bullous
pemphigoid
T
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Treatment
Topical steroids
Systemic steroids
20-40 mg/day
Dapsone
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Differences between pemphigus and
pemphigoid
Pemphigus Pemphigoid
Middle age Elderly
Flaccid blister Tense
Remains intact for short time Long
No other lesion preceding bullae Urticaria/eczema
General condition deteriorated Good
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DERMATITIS HERPETIFORMIS
Rare,
Intensely pruritic chronic,
Recurrent
Papulovesicular disorder
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Clinical features
Age - 20-50 yrs
Pruritus-first/predominant symptom
Early lesions-
papules, weals or grouped vesicles
Extensor aspects of body
Gluten sensitive enteropathy
DERMATITIS HERPETIFORMIS
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DERMATITIS HERPETIFORMIS
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Treatment
Gluten free diet
Dapsone :100-200 mg/day.