vesiculobollous disorders Flashcards
Auto-immune blistering disease of the skin & mucus membranes
pemphigus
histologic char of pemphigus
Intraepidermal blisters due to
acantholysis.
loss of intercellular connections, such as desmosomes,
resulting in loss of cohesion between keratin
Acantholysis
DIF vs Biopsy
in DIF sample besudes the blister or normal perilesional skin; Biopsy sample from early vesicle
Most superficial type of Pemphigus
PEMPHIGUS FOLIACEUS
Localized form of PF
Pemphigus erythematosus
presents with oral lesions in over 50% of patients. Oral lesions (buccal involvement) may be the only manifestation of the disease
PEMPHIGUS VULGARIS (PV)
epidermal separation in this disease occurs low in the epidermis
• “row of tombstone”
PV
Autoimmune blistering disease linked to a lymphoproliferative disorder (non-Hodgkins Lymphoma, Chronic Lymphocytic Leukemia)
PARANEOPLASTIC PEMPHIGUS
DIF in paraneoplastic pemphigus
intracellular epidermal IgG and C3
Recurrent vesicles and erosions of familiial benign pemphigus particularly are on
flexural
areas (submammary regions, inguinal folds, scrotum
Autoimmune subepidermal blistering diseases
pemphigoid
Subepidermal blistering disease; Large, tense pruritic blisters; Seen in flexural areas
BULLOUS PEMPHIGOID (BP)
DIF of BP
linear band of C3 and IgG on EBM
why BP has (-) N sign
bcoz it is deep down the dermis
Desmosomal plaque protein that
is important in anchoring keratin intermediate
filaments to the desmosome.
Desmoplakin 1:
elderly patient Large tense blisters (-) Nikolsky sign (+) pruritus Mucous membrane spared Flexural areas affected
BP
Rare, subepidermal blistering disease of the skin & mucous membranes
LINEAR IGA DERMATOSIS (LAD) & CHRONIC
BULLOUS DISEASE OF CHILDHOOD (CBDC)
cluster of jewel”, “collarette of blisters
LINEAR IGA DERMATOSIS (LAD) & CHRONIC
BULLOUS DISEASE OF CHILDHOOD (CBDC)
Immunohistopatholigic findings of LAD and CBDC
homogenous band of
IgA on the DEJ
DIF of LAD
Linear deposition of IgA
Rare, polymorphic inflammatory, subepidermal bullous
dermatosis of pregnancy (4th-7th month
PEMPHIGOID GESTATIONIS (PG) (HERPES GESTATIONIS
DIF of PEMPHIGOID GESTATIONIS (PG) (HERPES GESTATIONIS
linear deposit of C3 along DEJ
Intensely itchy chronic papulovesicular eruption
• Symmetric, tiny vesicles, papules, and urticarial plaques arranged in groups
DERMATITIS HERPETIFORMIS
DIF of Dermatitis herpetiformis
Granular PAPILLARY deposits of IgA
Vesicles and erosions of dermatitis herpetiformis are commonly found where
on the buttocks
biopsy of dermatitis herpetiformis shows
Subepidermal clefts with microabscesses of neutrophils and eosinophils in the dermal papillae
Rare, chronic auto-immune subepidermal blistering disease with erosive lesions
CICATRICIAL PEMPHIGOID
Conjunctiva and oral mucosa: most common areas affected by this
CICATRICIAL PEMPHIGOID
DIF of Cicatricial pemphigoid
Linear deposits of IgG and C3 on BMZ
