BP Ag2 (97-120 kDa) antigen
Linear IgA disease
Bullous pemphigoid antigens
Ag1: BP230 (intermediate filaments to HD plaque)
Ag2: BP 180 (transmembrane protein attaches to laminin 5)
Hemidesmosomal proteins
Herpes gestationes antigen
BP Ag2-NC16A antigen (less often BP Ag1)
Circatricial pemphigus antigen
BP Ag2, BPAg1, LAM 5, LAM 6
Junctional epidermolysis bullosa with pyloric atresia Antigen
Alpha6-beta4 integrity
Epidermolysis bullosa simplex with muscular dystrophy antigen
Plectin
Epidermolysis bullosa aquisita and Bullous LE Antigen
Collagen VII
Diseases with immunofluorescence on roof of salt split skin (4)
Bullous pemphigoid
Pemphigoid gestationis
Linear IgA bullous dermatosis
Cicatricial pemphigoid
Diseases with immunofluorescence on base of salt split skin
Anti-epilgrin cicatricial pemphigoid
Epidermolysis bullosa aquisita
Bullous LE
Defect in Collagen VII
EBA, DEB, Bullous SLE, CP
Name two cadherins in the skin
desmoglein and desmocollin
Name 5 members of the Plakin family
Desmoplakin, BPAg1, Plectin, Envoplakin, Periplakin
Name 2 other names for laminin 5
Epilegrin and Laminin 332
Where are the anchoring filaments located
Lamina Lucida
Where are the anchoring fibrils located
Sublamina densa
What is the predominant collagen in the Lamina densa
Type IV collagen
What is collagen which attaches Type I and III collagen to the Lamina densa
Type VII collagen
Name the tissue used for indirect IF for Pemphigus Vulgaris (and DH)
Monkey Esophagus
Name the tissue used for indirect IF for Paraneoplastic Pemphigus
Rat Bladder
Name the tissue used for indirect IF for Pemphigus Foleacius
Guinea Pig Esophagus
Name the medium used for transport of tissue used for DIF
Michel’s media
What is the sign that on slight rubbing the skin reveals the dermis?
Nikolsky sign
What is the sign: pressure on the blister surface results in lateral spread?
Asboe-Hansen sign
Where do blisters/erosions commonly first appear in pemphigus vulgaris?
Mouth
HLA associated with PV?
HLA DR4 and DR6 most commonly
The mucosal variant of PV is due to what antibody?
Anti-Desmoglein 3
The mucocutaneous variant of PV is due to what antibody?
Anti-Desmoglein 1 & 3
What do you see on pathology in PV?
Suprabasilar acantholysis with Tombstoning and eosinophilic spongiosis
What is the DIF pattern in PV?
Chickenwire with IgG4
What are the 2 subtypes of Pemphigus Vegetans?
Neuman (MC and severe), and Hallopeau (mild, pustular)
Does Pemphigus Foliaceus have oral lesions?
NO
Name drugs implicated in Pemphigus Foliaceus
Thiol-drugs (captopril, gold thiosulfate), sulfa-releasing, penicillamine, nifedipine
What’s the histology of PF look like?
Subcorneal/ Intragranular acantholysis with “cling ons”
In which country is endemic pemphigus found?
Brazil
What age population is involved in endemic pemphigus?
Children and young adults
What arthropod bite is possibly related to Fogo Selvalgem?
Simulium nigrimanum
What is another name for Pemphigus Erythematosus and what other disease does it share features with?
Senear-Usher syndrome and Lupus
What is the antibody in Pemphigus Erythematosus?
Anti-desmoglein 1
What are the antibodies in Paraneoplastic Pemphigus?
Plakin, BPAg1, Desmoplakin 1 and II, Envoplakin, Periplakin, alpha-2-macroglobulin-like-1
Name the associated malignancies with paraneoplastic pemphigus
NHL (40%), CLL (30%), Castleman’s disease (10%), Thymoma/Sarcoma/Waldenstrom’s (6% each)
What is the MC malignancy in adolescents with paraneoplastic pemphigus?
Castleman’s disease
What is the HLA associated with Paraneoplastic pemphigus?
HLA-DRB1
What is the most consistent symptom of paraneoplastic pemphigus?
Intractable Stomatitis
What process is associated with poor prognosis in paraneoplastic pemphigus?
Bronchiolitis obliterans
What are the 2 types of IgA pemphigus and their antibodies?
Subcorneal pustular dermatosis (desmocollin 1)
Intraepidermal neutrophilic dermatosis (Desmoglein 1/3)
What are the antibodies in Bullous Pemphigoid?
BPAg 1 (230kD) and 2(180kD)
What is the HLA assoc with BP?
HLA-DQB1
What % of BP have oral involvement?
20%
What is seen on DIF in BP?
Linear C3 and IgG4 at basement membrane
Where does the IIF localize to in BP?
Roof
Where does the BPAg2 Ab attack in BP?
NC16a domain
What common drug is known to cause BP?
Furosemide
What trimester does pemphigoid gestationis usually occur in?
2nd
What is the antibody in pemphigoid gestationis and what domain?
BPAg2 and NC16a domain
What disease is associated with Pemphigoid gestationis and HLA association?
Grave’s and HLA DR3 and DR4
What areas of the body are usually spared in PUPPP?
Periumbiical, upper chest, face, mucous membranes, palms and soles
What is the risk to mother and fetus in PUPPP?
None
What lab abnormality can Impetigo Herpetiformis be associated with?
Hypocalcemia (hypoparathyroidism)
Pregnant women with cholestasis of pregnancy are at risk for what process postpartum and why?
Postpartum hemorrhage due to low vitamin K (Fetus also at risk)
Cicatricial pemphigoid involves the conjunctiva and oral mucosa in what %?
66% and 90% respectively
Skin lesions are seen in what % in cicatricial pemphigoid?
25%
What is the name of cicatricial pemphigoid on the head and neck without oral involvement?
Brunsting-Perry
What is the antibody in cicatricial pemphigoid with mucosal and skin lesions?
BPAg2 (distal C-terminal)
Ab with ocular cicatricial pemphigoid?
Beta-4 integrin
Ab with malignancy associated cicatricial pemphigoid?
Laminin 332
What is the drug of choice for cicatricial pemphigoid with rapidly progressive eye disease?
Cyclophosphamide
What drug is ineffective for cicatricial pemphigoid with severe eye involvement?
Cyclosporine
What is the antibody against in Epidermolysis Bullosa Acquisita?
Type VII collagen (NC1 domain)
What is another name for Dermatitis Herpetiformis?
Duhring Disease
What areas of the body are involved in DH?
Extensor elbows/knees, scalp, and nuchal areas
What is the HLA association in DH?
HLA DQ2 and DQ8
What diseases are associated wit DH?
Thyroid disease (38-50%), small bowel T-cell lymphoma; DM, Addison’s, hepatitis, alopecia areata, MG, sarcoidosis, scleroderma, Sjogren’s, SLE, vitiligo
What is the appearance of the DIF in DH?
Granular IgA along DE junction
What are common antibodies are seen in DH?
Antiendomysial Ab, Antiepidermal transglutaminase Ab, Antigliadin Ab
What drug is commonly known to cause Linear IgA disease?
Vancomycin
What is the antibody seen in Linear IgA disease?
97-120kD epitope of BPAg2
What can flare Grover’s disease?
Heat or bedrest (hospitalization)