Vesiculobullous diseases BY Omar Abdullah

CLASSIFICATION OF VESICULOBULLOUS DISEASES INTRA EPITHELIAL VESICLES: The lesion is formed within the epithelium Acantholytic vesicles : This is because of the break down of specialized attachments called the desmosomes Nonacantholytic vesicles: It is usually in the viral infections because of the death or the rupture of the group of cells. SUB EPITHELIAL VESICLES: Lesions formed between the epithelium and the lamina propria eg: Erthyma multifome Phempegoid Dermatitis herpetiformis Epidermolysis bullosa

PEMPHIGUS VULGARIS A rare autoimmune disease. Common in Ashkenazi and Mediterranean jews . Middle aged and older. Other variants are: Pemphius vegetans Paraneoplastic pemphigus

PEMPHIGUS VULGARIS CLINICAL FEATURES: Painful erosions are formed in the oral cavity and skin. The bulla is rapidly ruptured leaving a collapsed roof of grayish membrane with a red ulcerated base. The ulcer may look like an apthous ulcer or may be large map shaped. Nikolsky sign is positive.

PEMPHIGUS VULGARIS Sometimes the ulcers are joined together to make a confluence. this condition is very painful. It has a variable course and might involve any mucous membrane as oesophagus, cervix. Protein/fluid,electrolyte and weight loss /secondary infections. Fatal if untreated.

PEMPHIGUS VULGARIS

PEMPHIGUS VULGARIS PATHOGENESIS: It is an autoimmune disease There are circulating antibodies of type IgG. These antibodies are reactive against the desmosomes or the tonofilament complex. There destruction or disruption of these tonofilament complex ,resulting in the loss of attachment from cell to cell

PEMPHIGUS VULGARIS HISTOPATHOLOGY Intra epidermal (suprabasilar) vesicles or bulla and cleft like spaces are produced (acantholysis). These changes are in the stratum spinosum or the prickle cell layer. Inflammatory cells are very scanty however eosinophils may be seen.

PEMPHIGUS VULGARIS histology

DIAGNOSIS Skin biopsy Electron microscopy has shown that widening of the intercellular space is followed by splitting of the desmosome junctions. Direct & indirect immunofluorescence ELISA

PEMPHIGUS VULGARIS DIFFRENTIAL DIAGNOSIS: Bullous Pemphegoid Erthema multiforme Bullous lichen plannus

PEMPHIGUS VULGARIS TREATMENT: High mortality rates Hospital admission Topical potent steroids Prednisolone plus azathioprine Rituximab Others

BULLOUS PEMPHIGOID Bullous pemphigoid is an affliction of elderly people,with onset usually after 60 years of age. The blister in bullous pemphigoid is subepidermal with an intact and often viable epidermis forming the roof. Bullous pemphigoid commonly starts with itching and a non-specific rash on the limbs that may be either urticaria-like or occasionally eczematous and rarely may simulate vesicular eczema.

PEMPHGOID Blisters may arise on erythematous and on normal skin and may be associated with dermal edema. The blisters are tense and dome shaped, obtaining a diameter of many centimeteres. The blisters are tough (Nikolsky sign negative) and may remain intact for several days, the contents often becoming jelly-like with coagulated fibrin. Mucosal lesions occur less frequently (25%) and are less severe than in pemphigus vulgaris and are usually confined to the mouth.

BULLOUS PEMPHGOID

BULLOUS PEMPHIGOID HISTOLOGY

PEMPHGOID Untreated bullous pemphigoid runs a chronic, self limiting course over a number of months or years. The disease duration is usually 3-6 years, with most patients achieving complete remission off treatment.

TREATMENT Topical and systemic steroids are the mainstay of treatment. For localized BP, very potent topical steroids are often sufficient. Antibiotics Systemic steroids Immunsuppressives IVIG Plasmapheresis