2. Vesiculobullous Disorders Flashcards
What are vesiculobullous disorders?
Heterogeneous group of disorders affecting the skin or mucous membranes
Vesiculobullous disorders may come as?
Blister
Vesicles
Blisters are?
Fluid-filled cavity within or beneath the epidermis
Vesicles are?
< 1 cm
Bullas are?
> 1 cm
What can blisters be secondary to?
Herpes infection
spongiotic dermatitis
burns
lupus erythematous
lichen planus
scleroderma
What are some key features for the diagnosis of vesiculobullous disorders?
*Level of plane of separation
*Presence or absence of acantholysis
*Characterization of inflammatory infiltrate
*Immunofluorescence pattern
*Intercellular bridges, a key morphological feature of keratinocytes
What are intercellular bridges?
a key morphological feature of keratinocytes
What are desmosomes?
a strong intercellular adhesion structure
What are hemidesmosome?
a strong adhesion structure between keratinocytes and basement membrane
What are the types of inflammatory blistering disorders?
Pemphigus
pemphigus vulgaris
Paraneoplastic pemphigus
Bullous pemphigoid
What are the types of non-inflammatory blistering disorders?
Epidermolysis Bullosa
Epidermolysis Bullosa Simplex
Epidermolysis Bullosa Dystrophic Type
Porphyria
Herpes
What are the 5 types of Pemphigus?
Vulgaris
Erythematosus
Foliaceus
Vegetans
Paraneoplastic
Pemphigus may be associated with?
internal malignancies, including thymoma
Intraepidermal vesicles are associated with?
acantholysis
What is the pathogenesis of Pemphigus?
Autoantibody, IgG, against desmoglein 1 and 3
(T/F) Pemphigus vulgaris is the most common type?
True
What areas are involved in pemphigus vulgaris?
BOTH mucosa and skin
Describe the fluid of pemphigus vulgaris
clear
What is usually accompanied with pemphigus vulgaris’ healing?
post-inflammatory hyperpigmentation
During pemphigus vulgaris, what may cause death?
staphylococcal infection
pulmonary embolism
Describe the pathological features of pemphigus vulgaris
Suprabasilar bullae with single row of basal cells resides on basement membrane
What are the clinical features of pemphigus vulgaris?
skin blisters filled with clear fluid
ruptured oral mucosa bullae appearing as ulcer
Pemphigus erythematosus is referred to ask what syndrome?
Senear-Usher
Pemphigus erythematosus appears where?
localized areas, resemble lupus malar rash on face, and may be considered as a localized less severe form of Pemphigus foliaceus
Paraneoplastic pemphigus is associated with what internal tumors?
lymphoma
thymoma
carcinoma of the bronchus, pancreas, and breast
Paraneoplastic pemphigus involves what areas
BOTH oral mucosa and skin
Describe bullous pemphigoid
Subepidermal, nonacantholytic, autoimmune disease
What antibodies are present with bullous pemphigoid?
IgG
IgG antibodies are with what hemidesmosoal proteins such as
BP230 and BP180
bullous pemphigoid is mostly seen in what population?
elderly
(T?F) Bullous pemphigoid involves the oral or ocular mucosa?
False…rarely
Does bullous pemphigoid rupture easily?
No
How does bullous pemphigoid heal?
without scarring unless infected
Epidermolysis Bullosa is?
a group of inherited disorders
When does Epidermolysis Bullosa primarily occur?
onset at birth or early infancy
How is eidermolysis Bullosa classified?
based on level of tissue separation
What form of Epidermolysis Bullosa is most common
simplex
Is Epidermolysis Bullosa simplex is autosomal dominant?
yes
Where are the mutations located in Epidermolysis Bullosa simplex?
keratin 14 or 5
What is the result of the degeneration of the basal layer in Epidermolysis Bullosa?
clinical bullae
What time of separation is present in Epidermolysis Bullosa simplex?
intraepidermal
What are the pathological findings of Epidermolysis Bullosa Simplex ?
Intact stratum corneum and upper epidermis, with vesicle formation in the lower epidermis at the basal layer caused by degeneration of individual epidermal cells
blisters are located where in Epidermolysis Bullosa Junctional Type?
lamina lucida
In Epidermolysis Bullosa Junctional Type, how may the skin appear?
normal
where is the defect located in Epidermolysis Bullosa Junctional Type?
laminin 5
Epidermolysis Bullosa Junctional Type is autsomal ______?
recessive
Epidermolysis Bullosa Dystrophic Type may be autosomal ____?
dominant or recessive
Where does Epidermolysis Bullosa Dystrophic Type blister?
beneath the lamina densa
Epidermolysis Bullosa Dystrophic Type has what pathological features?
o Few amount of anchoring fibrils beneath the lamina densa
What is Porphyria?
Non-inflammatory blistering disorder due to disturbance of porphyrin metabolism
What is the hereditary pattern of porphyria?
Autosomal dominant except congenital erythropoietic porphyria (autosomal recessive)
What is a clinical feature of porphyria?
o Delta-aminolefulinic acid (DALA): Pain
What is herpes?
Painful diseases caused by herpes simplex virus (HSV) or varicella zoster virus (VZV, also causes chickenpox)
What are the pathological features of herpes?
Grouped vesicles
Multinucleated cells with distinct nuclear inclusions
