Updated: 9/8/2017

Bullous Pemphigoid

Topic
Review Topic
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Questions
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Evidence
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Snapshot
  • A 75-year-old woman living in a nursing home presents with a 1-month history of severe pruritus. She reports having unrelenting pruritis with a pink rash over her trunk and extremities. Though she tried multiple lotions, the pruritus does not improve. Approximately 2 days ago, she also noticed some blisters on her body. She has never had rashes like this before. On physical exam, she has several 1-3 cm tense bullae on her abdomen and upper arms. Nikolsky sign is negative. A skin biopsy is taken and sent for immunofluorescence.
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
  • 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
      • typically symmetrically distributed on trunks and extremities
    • 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
  • Labs
    • autoantibodies in serum detected with enzyme-linked immunosorbent assay (ELISA)
      • for monitoring disease activity
      • anti-BP180 antibodies
      • anti-BP230 antibodies
  • Histology
    • subepidermal blister with eosinophils
    • direct immunofluorescence
      • linear pattern of IgG and C3 deposition along the epidermal-dermal junction
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
 

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Questions (3)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.MK.1) A 50-year-old female presents to her physician with vesicles and tense blisters across her chest, arms, and the back of her shoulders. Physical examination reveals that blistering is not present in her oral mucosa, and the epidermis does not separate upon light stroking of the skin. The patient most likely suffers from a hypersensitivity reaction located: Review Topic

QID: 101305
1

Linearly along the epidermal basement membrane

59%

(60/101)

2

In net-like patterns around epidermal cells

12%

(12/101)

3

In granular deposits at the tips of dermal papillae

15%

(15/101)

4

In fat cells beneath the skin

3%

(3/101)

5

In nuclei within epidermal cells

6%

(6/101)

M1

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