Updated: 6/23/2019

Bullous Pemphigoid

0%
Topic
Review Topic
0
0
N/A
N/A
Questions
4
0
0
Topic
Snapshot
  • A 80-year-old man presents with a 2-week history of severe pruritus and eczema. His past medical history includes atopic dermatitis, thyroid disease, and multiple sclerosis. On physical exam, he has dozens of 1-2 cm tense bullae and several erosions over his upper and lower extremities. His oral mucosa is not involved. Nikolsky sign is negative. A skin biopsy is taken and anti-BP180 is detected in his blood. A potent topical steroid is prescribed.
Introduction
 

 
  • Clinical definition
    • bullous pemphigoid (BP) is an autoimmune blistering disorder characterized by bullae (> 1 cm large, fluid-containing blister) and severe pruritus
  • Epidemiology
    • demographics
      • more common in those > 70 years of age
      • rarely in infants
  • Pathogenesis
    • auto-antibodies (IgG) against hemidesmosomes in the epidermal-dermal junction
      • antibodies are below the epidermis
    • main autoantigens are BP180 and BP230
    • this activates complement and inflammatory reaction which cause epidermal-dermal splitting
  • Associated conditions
    • drug use
      • loop diuretics
      • metformin
      • neuroleptics
    • neurologic conditions
      • multiple sclerosis
      • dementia
      • Parkinson disease
  • Prognosis
    • can resolve spontaneously
    • often recurs
Presentation
  • Symptoms 
    • severe pruritus
    • may have history of eczematous or urticarial lesions before bullae formation
  • Physical exam
    • tense bullae with clear exudate 
      • may be hemorrhagic
      • does not easily rupture
      • in adults
        • typically symmetrically distributed on trunks and extremities
      • in infants
        • palms and soles more commonly affected
    • spares mucous membranes
    • negative Nikolsky sign
      • cannot cause separation, blister formation, or blister extension with blunt pressure or lateral traction (dragging finger on skin)
    • may have vesicles
Studies
  • Based primarily on clinical presentation and observation
  • Skin biopsy
    • shows subepidermal blisters which are often with eosinophil-rich infiltrates
  • Immunofluorescence  
    • shows a linear band of immunoglobulins targeting hemidesmosomes and complement at epidermal-dermal junction
  • Complete blood count (CBC)
    • may show eosinophilia
Differential
  • Pemphigus vulgaris
    • more severe than BP
    • affects mucous membranes
    • positive Nikolsky sign
Treatment
  • Conservative
    • discontinue new medications
      • indications
        • if BP is suspected to be a drug reaction
    • wound care
      • indications
        • all patients
      • antiseptic care for erosions
  • Medical
    • topical corticosteroids 
      • indications
        • first-line treatment
        • often used with systemic treatments
      • drugs
        • clobetasol
    • prednisone
      • indications
        • for widespread disease or if topical steroids are not feasible
    • steroid-sparing immunosuppressant
      • indications
        • for those contraindicated to steroids
      • drugs
        • azathioprine
Complications
  • Skin and soft tissue bacterial infection of open lesions

Please rate topic.

Average 5.0 of 3 Ratings

Questions (4)
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK
Question locked
Sorry, this question is for
PEAK Premium Subscribers only
Upgrade to PEAK

(M2.DM.14.7) A 64-year-old man presents to the emergency department with a rash. He states the lesions are very pruritic and have been enlarging in size. His temperature is 97.6°F (36.4°C), blood pressure is 155/94 mmHg, pulse is 87/min, respirations are 12/min, and oxygen saturation is 98% on room air. The patient is admitted and a biopsy of the patient's skin lesions reveals linear IgG/C3 deposits along the area of the hemidesmosome. Which of the following is the most likely physical exam finding in this patient?

QID: 104218
FIGURES:
1

Figure A

8%

(3/38)

2

Figure B

11%

(4/38)

3

Figure C

53%

(20/38)

4

Figure D

13%

(5/38)

5

Figure E

8%

(3/38)

M 5 E

Select Answer to see Preferred Response

EXPERT COMMENTS (0)
Private Note