BMZ & Vesiculobullous Diseases Flashcards Preview

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Flashcards in BMZ & Vesiculobullous Diseases Deck (13):

Order of proteins in BMZ (descending)

Laminin 5
Collagen IV
Collagen VII


4 layers of BMZ & associated proteins (descending)

Hemidesmosome - BPAG 1 / 2 and integrins
Lamina lucida - traversed by BPAG2
Lamina densa - laminin 5 & collagen IV
Sublamina densa - collagen I, III, VII, and elastin (tensile strength)


Serologic tests of Desmoglein 1 and 3 indicative of?

Pemphigus vulgaris


Serologic tests of BPAG 1 and 2 indicative of?

Bullous pemphigoid


Pemphigus vulgaris (sxs, histology, DIF, antibodies, Nikolsky, tx)

Painful oral erosions
Flaccid bullae & erosions
Crusting of scalp
Row of tombstones (above basal layer)
DIF - IgG and C3 throughout whole epidermis
Antibodies against Dsg3
Positive Nikolsky
Tx - Systemic steroids & immunosuppressants


Bullous pemphigoid (sxs, split, DIF, antibodies, Nikolsky, tx)

Age 60+
Severe itching, but NOT painful
Tense bullae, oral mucosa more rare (25%)
Urticarial plaques or inflammatory papules
Splits at level of hemidesmosome (BELOW basal layer, epidermis completely detached from BMZ). Eosinophils may be present
DIF - linear deposition of C3 and IgG at BMZ
Antibodies against BPAG1 / 2
Negative Nikolsky
May resolve on own, topical steroids, oral antibiotics (tetracycline), oral steroids / immunosuppresants for severe disease.


Cicatricial pemphigoid (sxs, histology, DIF, antibodies, Nikolsky, Tx)

Oral erosions (dysphagia)
Ocular disease - burning, pain, conjunctival scarring, ectropion (lower eyelid sags), symblepharon (conjunctivae fuse) decreased acuity
Cutaneous blisters more rare (25%) - plaques, bullae, atrophic scarring
Tense bullae
Subepidermal blister
DIF - linear IgG and C3
Antibodies against BPAG2 and laminin 5
Negative Nikolsky
Tx - mild: topical corticosteroids. Severe: oral immunosuppressants & ophthalmologic exams.


Congenital mechanobullous diseases (presentation, cause, workup, and tx)

Vesicles, bullae, and erosions
Absence of BMZ structural proteins
Workup - biopsy and electron microscopy
Tx - avoidance of mechanical trauma & wound care


Epidermolysis Bullosa (EB) Simplex
Inheritance, sxs, and abnormal component

Autosomal dominant
Blisters over hands, feet, extremities
Absent epidermal keratins


Junctional EB
Inheritance, sxs, and abnormal component

Autosomal recessive
Widespread blistering, mouth / esophageal lesions
Laminin & BPAG2 mutations


Dystrophic EB
Inheritance, sxs, and abnormal component

Autosomal recessive & dominant
Blisters that heal w/ scarring
Mitten-like deformities to hands (digits start to fuse together)
Abnormal / absent collagen VII


Dermatitis Herpetiformis (location, sxs, associated sx, histology, DIF, Serum, tx)

Extensor upper / lower extremities, butt, and occipital scalp
Intensely itchy, so intact vesicles rarely seen
Vesicles, papules, & urticarial plaques
Related to celiac disease, gastric lymphoma, and thyroid disease
Subepidermal bullae w/ neutrophils
DIF - IgA tips of dermal papillae
Serum - anti-tissue transglutaminase IgA
Tx - gluten free diet and dapsone


Immunobullous diseases (presentation, cause, tx)

Arise from inflammation
Vesicles, bullae, and erosions
Antibodies against BMZ
Tx - topical steroids, oral antibiotics, and immunosuppressants