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BMZ and Vesiculobullous Diseases (Mechanobullous Diseases (Treatment…
BMZ and Vesiculobullous Diseases
BMZ
Cellular Components
BPAG 1 and BPAG 2
Functions of BMZ
connects the basal layer of the epidermis to the dermis
extracellular condensation of mucopolysaccharides and proteins between the epithelial and connective tissue
anchors proteins that transwerve the region between the epithelial and connective tissue
Ddx the Diseases of the BMZ
Diagnostic testing needs to be used:
skin biopsy, DIF and IIF, serological tests all used to distinguish between the blistering diseases
Use of Clinical lesion appearance for vesculobullous
Size
: vesicles vs bullae
Quality
: flaccid vs tense
Nikolsky sign
: positive means that it's in the epidermis vs negative's sign means its in the BMZ, dermal
Immunobullous Disease
Evaluation
skin biopsy:
need the tissue specimen to include the edge of the blister (X) for histopathology and then include the peri-lesional skin (y) which is >1 cm from the edge to use for DIF
DIF and IIF
DIF: detects immuinoreactants causing the blisters
1) create a tag to find it. Take anti-human antibodies from a mouse to detect the presence of antibodies against the skin
2)take probe to a perilesion and look for the linkage fo the two antibodies and detect through a flurosence microscope
3) bands will tell us whether antibodies are present in the skin
IIF: the patient's serum is tested for the presence of autoantibodies
histopathology:
localizes the level of the spilt and identifies the inflammatory cell type
Serology:
ELISA for antigen levels
Dsg 1&3 antigens - pemphigus vulgaris
BPAG 1&2 antigen - bullous pemphigoid
Elevated levels means that the disease is still active
Types
Bullous Pemphigoid
Cicatricial Pemphigoid
Pemphigus Vulgaris
Treatments
Oral and topical corticosteroids (taper slowly)
Oral antibiotics: needed ONLY for BP
General: BP may resolve over time however PV and CP could have a high morbidity/mortality if untreated
Oral immunosuppressants
Dermatitis Herpetiformis:
Clinical presentation: cutaneous lesions that are prussic and rarely found intact as vesicles oue to scratching
Treatment: gluten free diet, always associated with celiac disease
Evaluation
sub epidermal blister with neutrophilic response
DIF: IgA granular deposits in dermal papillae
Serum: anti-endomysial antibodies
Mechanobullous Diseases
Structural Causes: congenital absence of BMZ structural proteins
Clinical Features:
Skin fragility, painful blistering and scarring where the level of skin involvement can determine the disease severity and degree of functional impairment
Evaluation
Diagnosis occurs with immunohistochemical stains of skin biopsies OR electron microscopy
Types
Epidermolysis Bullosa
Junctional EB (AR)
EB simplex (AD)
Dystrophic EB (both)
Treatment
Wound care
Avoidance of mechanical trauma