First Aid, Chapter 8, Immunologic Disorders, Immunologic Skin Disease Flashcards
What are the target skin structures for pemphigus?
Desmogleins-1 and -3 in desmosome for pemphigus
What are the target skin structures for pemphigoid and linear IgA bullous dermatosis?
BP Ags (BP180 and BP230) in hemidesmosome/lamina lucida for pemphigoid and linear IgA bullous dermatosis.
What are the target skin structures for epidermolysis bullosa acquisita?
Type VII collagen or anchoring fibrils for epidermolysis bullosa acquisita
What are the clinical signs of pemphigus?
Flaccid bullae on noninflamed skin Nikolsky’s sign Found on scalp, chest, oral mucosa, intertriginous areas
What is the target of pemphigus vulgaris?
IgG epithelial surface Desmoglein-3 +/– desmosome
What is the immunopathology of pemphigus vulgaris?
Epidermal IgG/C3
What is the treatment of pemphigus vulgaris?
prednisone +- immunosuppresives
What is the target of pemphigus folicaceous?
Desmoglein-1 +/– desmosome
What is the immunopathology of pemphigus foliaceous?
Epidermal IgG/C3
What is the treatment of pemphigus foliaceous?
Steroids
What is the target of IgA pemphigus?
IgA epith surface Desmocollin-1
What is the immunopathology of IgA pemphigus?
Epidermal IgA
What is the treatment of IgA pemphigus?
Steroids
What are the clinical findings of bullous pemphigoid?
Tense bullae on urticarial base
Pruritus
Flexural areas
Elderly
What is the target of bullous pemphigoid?
IgG basement membrane zone (BMZ)
BP180
BP230
What is the immunopathology of bullous pemphigoid?
IgG/C3 (BMZ)
What is the treatment of bullous pemphigoid?
Steroids
Immuno-suppressives +/–
What are the clinical signs of herpes gestationis?
Urticaria Blistering Periumbilical area During or immediately postpartum May flare w OCP/menses
What is the target of herpes gestationis?
IgG BP180 BMZ
What is the immunopathology of herpes gestationis?
Linear BMZ C3
What is the treatment of herpes gestationis?
Steroids
Antihistamines
What are the affected areas of epidermolysis bullosa acquisita?
Areas of trauma
Oral mucosa
What is the target of epidermolysis bullosa acquisita?
Type VII collagen
What is the immunopathology of epidermolysis bullosa acquisita?
Linear BMZ IgG/C3
Linear IgA/IgM
What is the treatment of epidermolysis bullosa acquisita?
Colchicine or dapsone + steroids
Immuno-suppressives +/ – for refractory cases
What are the clinical signs of dermatitis herpetiformis?
Small grouped vesicles LE/buttocks
Intense pruritus
Associated with celiac disease
What is the target of dermatitis herpetiformis?
Endomysial IgA Transglutaminase IgA Epidermal transglutaminase
What is the immunopathology of dermatitis herpetiformis?
Granular BMZ IgA
What is the treatment of dermatitis herpetiformis?
Eliminate gluten from diet
What is the mnemonic for the key features of pemphigus?
DEsMO (Desmosome) = DEMO.
DEMO is on the surface (of skin); so, when DEMO is affected, the surface of skin is damaged, leading to flaccid bullae.
A 70-year-old woman presents with a pruritic rash over her upper extremities with what appear to be tense, fluid-filled blisters. What is the diagnosis?
Bullous pemphigoid. Tends to occur in the elderly, is pruritic, and occurs in flexural areas
What does tissue immunofluorescence show for lupus erthematosis?
Granular immune deposits at BMZ in both involved and uninvolved skin (lupus band), IgM = C3 >IgG >IgA, where two or more required. Refer to Tables 8-25 and 8-29 for antigen testing in these conditions.
