Pemphigus Flashcards
Subtypes of Pemphigus
Pemphigus Vulgaris
- Mucosal Dominant
- Mucocutaneous
- Pemphigus Vegetans
Pemphigus Foleacous
- inc Fogo Selvagem
Paraneoplastic Pemphigus
Pemphigus Erythematosus
IgA Pemphigus
Histology Findings Pemphigus Vulgaris
Intraepidermal Blister
Suprabasilar acantholysis (without keratinocyte necrosis)
- acantholysis extends down hair follicles
Epidermal cells and eosinophils within the blister cavity
Perivascular mononuclear infiltrates with eosinophils
Dermal papillae are maintained
DIF findings pemphigus Vulgaris
IgG +/- C3 intercellular staining - giving a chickenwire appearance.
Staining may be more prominent are the basilar / supreabasilar layer compared to the granular layer
IF findings for pemphigus vulgaris
Best demonstrated on Monkey Eosophagus
IgG staining throughout the epidermis
ELISA results of pemphigus vulgaris
DSG 3 +/- DSG 1
Histopathology of pemphigus vegetans
Similar to P. Vulgaris
- intraepidermal blister, acantholysis, eposinophils
With acanthosis and papilomatosus
More intense inflammatory cell infiltrate continaing eosinophils
Pemphigus Foliaceous Histology findings
Acanthosis at the level of teh granular layer
(often wihtout keratinocyte necrosis)
Stratum corneum often lost
Eosinophils and neutrohils
Mononuclear perivascular infiltrate with eosinophils
What are the DIF findings with Pemphigus Foliaceous?
Intercellular staining of IgG +/- C3 - chickenwire apeparance
More prominent at the stratum corneum / granulosum compared to the basal layer
IF findings for Pemphigus Foliaceous?
Human skin OR Guinea Pig eosphagus
Intercellular IgG staining +/- C3
Elisa results of Pemphigus Foliaceous?
DSG 1
Paraneoplastic pemphgigus most common malignancies?
Non-hodgkins lymphoma
CLL
Castlman disease
Paraneoplastic pemphigus histology findings?
Variable findings - intraepidermal blister formation / acantholysis
Oft ass wtih lichenoid inflamation - band like lymphocytic infiltarte abuting the dermo-epidermal junction
Basal vacuolar change and keratinocyte necorsis
DIF findings for paraneoplastic pemphigus
intercellular staining of IgG +/- C3
+ Prominent stainng along the dermoepidermal junction
ELISA findings for paraneoplastic pemphigus?
Envoplakin
Periplakin
Desmocollin 1 / 3
BP 230
IF findings for paraneoplastic pemphigus?
Best demonstrated on Monkey or guinea pig esophagus or rat bladder
IgG
intercellular staining of IgG +/- C3
AND
Prominent stainng along the dermoepidermal junction
Histology findings in pemphigus erythematosus?
Intraepithelial blister formation / acantholysis around the granular layer
Eosinophils
Plus lichenoid inflammation:
- band like perivascular inflammaiton
- mucin
- basal vaculoar change
- keratinocyte necorsis
What are the DIF findings of pemphigus erythematosus/
Intercellular deposits of IgG and C3 in the epidermis
+ granular staining of IgG and/or C3 along the dermal-epidermal junction
What are the histology findigns of IgA pemphigus?
Intraepidermal pustule or vesicle
- Predominant cell type = neutrophils
- May be subcroneal or intraepidermal
No acantholysis
What are the DIF findings of igA pemphigus?
IgA +/- C3 positive granular and intercellular, resembling a “chicken wire” pattern
On keratinocyte cell surfaces
IgA pemphigus has what associations?
Inflammatory bowel disease
IgA myeloma
Paraneoplastic Pemphigus is ass. with which conditions?
- Malignancy - Non-hodgkins lymphoma, CLL, castelmans, Sarcoma etc
- Benign tumours - e.g Thymoma
- Bronchiolitis Obliterans
What drugs are commonly associated with pemphigus?
ACEi
Penicillamines
Gold
Pyritinol
Antibiotics (penicillins)
ICIs (pembrolizubmab)
Topical Imiquimod
Propranolol
Subtuypes of IgA pemphigus? and the associated antigens?
Subcorneal pustular dermatosis type - Desmocollin 1
Intraepidermal neutrophillic type - unknown antigen
PF type - DSG1
PV type - DSG3
Classification of pemphgus
