BMZ & Vesiculobullous Diseases

Question 1

Order of proteins in BMZ (descending)

Answer 1

Hemidesmosome
BPAG1
BPAG2
Laminin 5
Collagen IV
Collagen VII

Question 2

4 layers of BMZ & associated proteins (descending)

Answer 2

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)

Question 3

Serologic tests of Desmoglein 1 and 3 indicative of?

Answer 3

Pemphigus vulgaris

Question 4

Serologic tests of BPAG 1 and 2 indicative of?

Answer 4

Bullous pemphigoid

Question 5

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

Answer 5

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

Question 6

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

Answer 6

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.

Question 7

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

Answer 7

Pain
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.

Question 8

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

Answer 8

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

Question 9

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

Answer 9

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

Question 10

Junctional EB

Inheritance, sxs, and abnormal component

Answer 10

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

Question 11

Dystrophic EB

Inheritance, sxs, and abnormal component

Answer 11

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

Question 12

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

Answer 12

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

Question 13

Immunobullous diseases (presentation, cause, tx)

Answer 13

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