gambaran vesikobulosa

7/25/2019 gambaran vesikobulosa

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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



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Treatment

Gluten free diet

Dapsone :100-200 mg/day.

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gambaran vesikobulosa