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68 Cards in this Set
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- Back
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What d/o has DIF with IgG and complement within the intercellular spaces (Swiss cheese pattern)?
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Pemphigus foliaceus
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Antibodies to desmoglein III?
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Pemphigus vulgaris
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Name the 7 variants of Pemphigus
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1. Pemphigus vulgaris
2. Pemphigus vegetans 3. Pemphigus foliaceus 4. Pemphigus erythematosus 5. Paraneoplastic pemphigus 6. IgA pemphigus 7. Fogo selvagem |
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"dilapidated brick wall"?
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Hailey-Hailey
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Hailey-Hailey aka?
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Benign familial pemphigus
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Grover's Disease aka?
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transient acantholytic dermatosis
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What are the antibodies directed to in Bullous Pemphigoid?
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BP1 antigen (230kD, hemidesmosome desmoplakin 1 and 2) or
BP2 antigen (180kD, type XVII collagen, both hemidesmosome and lamina lucida) |
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Describe immunofluorescence in Bullous Pemphigoid
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Linear IgG (usu IgG4) and complement deposits at the basement membrane zone in the lamina lucida
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Size of Desmoglein 1
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160kD
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Size of BP1
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230 kD
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size of BP2
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180 kD
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Causes of sub/intracorneal bullas
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Bullous Impetigo
SSSS Pemphigus Foliaceous Pemphigus Erythematosus IgA Pemphigus Subcorneal Pustular Dermatosus AGEP -- Acute Generalized Exanthematous Pustulosis Miliaria Crystallina |
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Another name for Pemphigus Erythematosus
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Senear-Usher
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Intraepidermal disorders
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Spongiotic & Viral d/o
EB (Weber-Cockayne) Friction Blister |
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Suprabasilar
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Pemphigus Vulgaris
Pemphigus Vegetans Hailey-Hailey Darier's Grover's |
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Subepidermal (Cell-Poor)
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Epidermolysis Bullosa
Porphyria Cutanea Tarda |
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Subepidermal (w/ Eos)
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Bullous Pemphigoid
Herpes Gestationis |
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Subepidermal (w/Neuts)
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Dermatitis Herpetiformis
Linear IgA Cicatricial Pemphigoid |
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Miscellaneous Bullous d/o
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Paraneoplastic Pemphigus
Coma Blister |
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Three types of Infantile Pustular Dermatosis
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Infantile Acropustulosis
Erythema Toxicum Neonatorum Transient Neonatal Pustular Melanosis |
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Two types of IgA Pemphigus & their targets
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Subcorneal Pustular Dermatosis (SPD) - Desmocollin
Intra-Epidermal Neutrophilic (IEN) - Desmoglein 1 or 3 |
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DIF for Pemphigus Erythematosus
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DIF: intercellular "fish net" staining, plus linear band along DEJ
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What has "crushed cornflake" appearance?
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Pemphigus Foliaceus
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What is the target of Pemphigus Foliaceus?
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Desmoglein 1
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What has "kling on" cells?
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Pemphigus Foleaceus
found on blister roof DIF: intercellular IgG and C3 in upper epidermis |
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Subcorneal Pustular Dermatosis aka?
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Sneddon-Wilkinson
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Target of Pemphigus Vulgaris
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Desmoglein 3
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Pathology of Pemphigus Vulgaris
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"tombstoning" effect of the "villi" projecting into the blister cavity due to hemidesmosomes
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DIF of Pemphigus Vulgaris
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IgG & C3 in a "fishnet" or "chicken wire" pattern
IIF: monkey esophagus |
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Name the two types of pemphigus vegetans
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Neumann - vesicular/erosive evolving into vegetative
Hallopeau - pustular lesions, benign course |
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Gene for Hailey-Hailey
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ATP2C1 (AD)
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Path for Hailey-Hailey
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aka "benign familial pemphigus"
"dilapidated brick wall" Negative DIF |
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Gene for Darier's Disease
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ATP2A2 (AD; SERCA-2 Ca2+ ATPase)
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aka Dariar's dz?
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Keratosis follicularis
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What is Grover's Dz?
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idiopathic acneiform to pustular eruption on the trunk;
histo looks like PV, Hailey-Hailey, or Darier's |
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What are the three important forms of EB
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EB simplex
Junctional EB Dystrophic EB (fourth one -- EB Acquisita, rare) |
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Where is the split in most EBs?
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Subepidermal blister (except Weber-Cockayne, which is in the mid-upper epidermis)
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Target of EB Acquisita
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Collagen VII (290kD)
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What is EB Acquisita assoc with?
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SLE, RA, Crohn's, Thyroiditis, HIV
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Defect in EB Simplex?
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K5 & K14 defect (AD)
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What are the variants of EB Simplex?
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Dowling-Meara -- herpetiform grouped vesicles
Weber-Cockayne -- hands and feet Koebner -- generalized Muscular Dystrophy (EBS-MD) -- plectin defect |
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What is defective in Muscular Dystrophy (EBS-MD) variant of EB
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Plectin
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Defect in Junctional EB?
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Laminin 5 or BP2 (type XVII collagen)
AR defect |
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What are the three types of Junctional EB?
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GABEB -- generalized atrophic benign EB, non-Herlitz, Mitis), can get multiple SCCs later in life
Herlitz -- (Gravis, EB letalis): die in infancy Pyloric Atresia (JEB-PA): alpha-4-Beta-6-integrin defect |
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What is the defect in Dystrophic EB?
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collagen VII defect (same as in EBA); mitten deformities seen, SCC in 10-40%
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What are the variants of Dystrophic EB?
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Hallopeau-Siemens (AD)
Non-Hallopeau-Seimens (AR): Cockayne-Touraine has acral, hypertrophic scars and Pasini which has "albopapuloid" lesions on the trunk |
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Gene defect in Porphyria Cutnea Tarda
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Uroporphyrinogen decarboxylase
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What dz is assoc with PCT?
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Hep C
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What is the "Soret" band light range?
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UV 400-410nm
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Histo of PCT?
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"festooning": preservation of the dermal papillae
"caterpillar bodies": PAS+ hyaline bodies in roof hyalinized blood vessels in dermis |
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Target of BP?
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BP1 (230kD) and BP2 (180kD), type XVII collagen
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What two clinical signs are ABSENT in Bullous Pemphigoid?
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Nikolsky and Asboe-Hansen
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What HLA is assoc with BP?
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HLA-DQ7
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DIF in BP?
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linear IgG4 & C3 at BMZ
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Target of Herpes Gestationis?
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BP2 (180kD)
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Target in Dermatitis Herpetiformis?
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Tissue Transglutaminase
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Assoc HLA in DH?
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HLA DQ2, DR3, B8
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What antibodies are found in DH?
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IgA anti-endomysial, gliadin, antireticulin Abs
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Path of DH?
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papillary microabscesses "papillitis"
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Target of Linear IgA?
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LAD-1 (97kD)
ladinin-a breakdown product of BP2 |
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Linear IgA in children is known as
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Chronic Bullous Dermatosis of childhood
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Clinical picture in Linear IgA dermatosis?
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"crown of jewels"
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DIF in Linear IgA dermatosis?
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Linear IgA at DEJ
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Target in Cicatricial Pemphigoid?
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BP2 (180kD)
Epiligrin (alpha-3 subunit of laminin 5; increased cancer risk with this variant) Beta-4 integrin (ocular variant) |
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What is Brunsting-Perry?
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localized variant of cicatricial pemphigoid involving skin of head and neck w/o mucosal invovlement
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Target of Paraneoplastic Pemphigus?
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Desmoplakin (250kD)
BP1 (230kD) Periplakin (190kD) Envoplakin (210kD) |
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What IIF is positive in Paraneoplastic Pemphigus?
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positive on rat bladder epithelium
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What stains on salt-split skin in cicatricial pemphigoid?
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roof stains in the BP variant and the ocular variant; the epiligrin variant stains the floor
DIF: Linear IgG/C3 along BMZ |
