Vesiculobullous diseases

PEMPHIGUS VULGARIS PEMPHGOID ERYTEMA MULTIFORME EPIDERMOLYSIS BULLOSA LUPUS ERYTHEMATOSIS

Dr.khurram zafar

CLASSIFICATION OF VESICULOBULLOUS DISEASES

VESICLE&BULLA A clear fluid lesion just below the epithelium which ruptures to form an ulcer, if this is smaller than 5mm then it is a vesicle ,if larger than 5mm than it is a bulla

CLASSIFICATION OF VESICULOBULLOUS DISEASES

CLASSIFICATION 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
Autoimmune disease. Common in Ashkenazi and Mediterranean jews . Middle aged females. Other variants are: Pemphius Vegitans Pemphigus Foliaceus & Erthematosus Paraneoplastic pemphigus.

PEMPHIGUS VULGARIS
CLINICAL FEATURES:
Painful ulcers or bulla are formed which are fluid filled. They can be formed any where in the oral cavity . 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.
C/F cond

PEMPHIGUS VULGARIS
Some time the ulcers are joined together to make a confluence this condition is very painful. It has a variable course might involve skin, 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
path.contd

PEMPHIGUS VULGARIS
The epithelial damage is directly proportion to the number of the circulating antibobies. The tonofilament or desmosomes are disrupted by a proteolytic enzyme which is released by these antibodies . The cell to cell break down also takes place through a complement system but this process is not clearly understood .

PEMPHIGUS VULGARIS

PEMPHIGUS VULGARIS
HISTOPATHOLOGY: Intra epithelial vesicles or bulla and cleft like spaces are produced by acantolysis These changes are in the stratum spinosum or the prickle cell layer The basal cell remain attach to the lamina propria and project into the bulla like tombstones. Inflammatory cells are very scanty however eosinophils may be seen. Acantholytic statum spinosum cells occur singly or are in the forms of clumps lying freely within the blister fluid. These cell loose there polyhedral morphology rather they are small rounded and contain hyper chromatic nuclei called the TAZANK CELLS.

PEMPHIGUS VULGARIS
histology

PEMPHIGUS VULGARIS
histology

PEMPHIGUS VULGARIS
tazank cells

PEMPHIGUS VULGARIS
immunoflorecence

PEMPHIGUS VULGARIS
DIFFRENTIAL DIAGNOSIS: Pempegiod Erthema multiforme Bullous lichen plannus

PEMPHIGUS VULGARIS
TREATMENT: High mortality rates previously Introduction of systemic corticosteroids like prednisolone in stable cases. Prednisolone plus azathioprine methotrexate and cyclophospamide in progressed or advance cases.

PEMPHGOID

PEMPHGOID
Mucous membrane pemphigoid (cicatricial) CIKA-TRI-CIAL Bullous pemphigoid

PEMPHGOID
PATHOLOGY Autoimmune disease Not life threatening Elderly females above 60 yrs of age Loss of attachment and separation of full thickness epithelium from the lamina propria. Alteration of rete pegs Epithelium forms the roof of the blisters Auto antibodies are formed against the hemidesmosomes (BPAG-1,230kd;BPAG-2; 180kd. Inflammatory cells(lymphocytes,neutrophils,eosinophils)are seen in the later stages

PEMPHGOID

PEMPHGOID
CLINICAL FEATURES(MMP) Oral mucosa is the first site- lesions are rarely wide spread Subepithelial bullae, ruptured in the later stages. Bleeding in the bullae bleeding blisters Slow progress, skin involvement absent or rare Involvement of eyes, nose larynx, pharynx and osephaghus Nikolsky sign is positive

PEMPHGOID
occular involvement

PEMPHGOID
CASCADEOF EVENTS Antibody antigen complex Complement activation Neutrophils & Eosinophils recruited Release of proteases by the recruited cells Sub epithelial blister formation

PEMPHGOID

PEMPHGOID
MANAGEMENT Confirm diagnosis Topical corticosteroids Ocular involvement systemic steroids.

ERYTEMA MULTIFORME

ERYTEMA MULTIFORME
Mucocutaneous disease Males adolosents , young adults are affected more

ERYTEMA MULTIFORME
AETIOLOGY /PATHOLOGY Unclear aetiology and pathogenesis Infections like HSV can trigger this disease Drugs like Sulphonamides ,barbiturates Suggested cause is also given as to a type III hypersensitivity reaction

ERYTEMA MULTIFORME
CLINICAL FEATURES Prodomal signs: Upper respiratory infection Headache and malaise Nausea and arthralgia

C/Fcontd

ERYTEMA MULTIFORME
Signs during the disease: Red macules 1cm or more in diameter with cyanotic center Lips grossly swollen ,split crusted bleeding Widespread fibrin covered erosions and erythema in the mouth. Mild fever Conjunctivitis may be associated Attacks recur at the intervals of several months Usually self limiting.

ERYTEMA MULTIFORME

ERYTEMA MULTIFORME
HISTOPATHOLOGY Necrosis of the kertinocytes Inter & intra cellular odema. Subepithelial blisters are common Infiltration of inflammatory cells.

ERYTEMA MULTIFORME

ERYTEMA MULTIFORME
MANAGEMENT No specific treatment required , if HSV inf.. acycovir Systemic steroids may give relief to the fever. In severe cases antibiotics are used to prevent ant secondary infections. Symptomatic analgesics, antipyretics, antihistamines.

EPIDERMOLYSIS BULLOSA

EPIDERMOLYSIS BULLOSA
Definition: A large group of clinically similar desquamating disease processes of the skin and mucosa that have in common the separation of the epithelium from the underlying connective tissue and the formation of large blisters that frequently result in extensive and often immobilizing scar formation.

EPIDERMOLYSIS BULLOSA
MAJOR CATEGORIES OF EPIDERMOLYSIS BULLOSA Type Genetic Pattern Separation Level Hereditary Simplex Autosomal dominant Intraepithelial Junctional autosomal recessive lamina lucida Dystrophic autosomal dominant sublamina densa Defec. Structure linking proteins anchoring filaments type VII collagen

Acquired Acquisita

None/autoimmune

sublamina densa

type VII collagen

EPIDERMOLYSIS BULLOSA
HEREDITARY TYPES: Congenital absence of components ACQUIRED TYPES: Autoantibodies (IgG; sometimes IgA) to type VII collagen.

EPIDERMOLYSIS BULLOSA

EPIDERMOLYSIS BULLOSA
CLINICAL FEATURES 1.Epidermolysis Bullosa Simplex Mild form; autosomal dominant Sites of trauma/friction Involve hands, feet and neck; occ. knees and elbows Teeth not affected; intraoral blisters seen Appears during infancy

EPIDERMOLYSIS BULLOSA

EPIDERMOLYSIS BULLOSA
2. Junctional Epidermolysis Bullosa Severe form; autosomal recessive Haemorrhagic blisters; loss of nails, large blisters of face, trunk and extremities Generalized scarring and atrophy Intraorally-haemorrhagic blisters of palate, perioral and perinasal areas Erupted teeth exhibit hypoplastic and severely pitted enamel prone to caries

EPIDERMOLYSIS BULLOSA
3. Dystrophic Epidermolysis Bullosa Both autosomal dominant and recessive; recessive is severe Lesions are birth; arise at pressure sites Blisters rupture leaving painful ulcers which heal with large scars that undergo contractures, leading to loss of motility and claw-like hands (Mitten Deformity) Teeth exhibit delayed eruption and enamel hypoplasia with rapid caries development Scarring around mouth leads to diminished opening, ankyloglossia

EPIDERMOLYSIS BULLOSA
Epidermolysis Bullosa Acquisita Non-hereditary form; appears in adulthood Clinically resembles autosomal dominant type of JEB-type VII collagen Trauma/friction induced blisters of knees, elbows, hands and feet- heal with scars Intraoral blisters rare- when present same picture same picture as JEB

EPIDERMOLYSIS BULLOSA
HISTOPATHOLOGY Simplex type exhibits zone of cleavage (intra-epithelial) above basal cell layer. Remaining types have sub-epithelial separation

EPIDERMOLYSIS BULLOSA

EPIDERMOLYSIS BULLOSA
MANAGEMENT No specific treatment available for hereditary types Acquired form maybe treated with corticosteroids and immuno-suppressants Maintenance of pts nutritional and oral hygiene status Wound healing techniques Prevention of infections Systemic use of Phenytoin (also acts as a collagenase inhibitor)