Rovner

BULLOUS DISORDERS

1. Epidermolysis Bullosa--noninflammatory inheritable blistering disorders,skin fragility
1. E.B. Simplex
2. E.B. Letalis (junctional)
3. E.E. Dystrophica
4. Treatment: trauma avoidance
1. cool environs
2. antibiotics, local wound care
3. steroids
4. Dilantin
2. Pemphigus
1. Superficial
1. P. foliaceous
2. P. erythematosus
2. Deep
1. P. vulgaris
2. P. vegetans
3. flaccid bullae on uninflamed base
4. + Nikolsky sign
5. Pathogenesis: autoantibodies to intercellular cement
6. Histopathology
1. acantholysis
2. intrerepidermal bullae
3. + immunofluorescence intracellularly in epidermis
7. Treatment:
1. Steroids!
2. immunosuppressants
3. Bullous Pemphigoid
1. Tense bullae on inflamed base
2. Nikolsky sign
3. Pathogenesis: autoantibodies to basement membrane zone protein
4. Histopathology
1. no acantholysis
2. intact epidermis overlying bulla with eo's, PMN's
3. + immunofluorescence at DE junction
5. Treatment: Steroids, immunosuppressants
4. Dermatitis Herpetiformis
1. Intensely pruritic papulovesicles over elbows, knees, buttocks
2. gluten enteropathy (asymptomatic)
3. Pathogenesis: IgA antibodies in dermal papillae
4. Histopathology: Necrosis of dermal papillae with PMN's
5. Treatment: Dapsone, gluten free diet, antihistamines
5. Porphyria Cutanea Tarda
1. Blisters on dorsal hands with sun exposure
2. Zygomatic hypertrichosis
3. Marked skin fragility
4. Pathogenesis:
1. defect in uroporphyrinogen decarboxylase
2. increased porphyrins excreted in urine (positive fluorescence)
5. Aggravated by BCP's, EtOH
6. Treatment:
1. phlebotomy
2. antimalarials
3. EtOH avoidance
4. sunscreens