Updated: 11/29/2018

Pemphigus Vulgaris

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Snapshot
  • A 45-year-old woman presents to the physician's office for a painful and blistering rash. She reports having a 1-month history of nonhealing ulcers in her mouth, which she previously attributed to stress. However, she reports new ulcers in her mouth and new easily-ruptured blisters on her skin. Approximately 6 weeks ago, she reported starting a new drug, captopril, for management of her hypertension. On physical exam, she has dozens of flaccid bullae and erosions on her trunk and extremities; she has 3 ulcers along her buccal mucosa. She is started on systemic steroids and captopril is discontinued. 
Introduction
 

 
  • Clinical definition
    • pemphigus vulgaris (PV) is a potentially fatal autoimmune blistering disease characterized by painful mucocutaneous lesions
  • Epidemiology
    • demographics
      • most common in
        • those of Mediterranean or Jewish descent
        • 40-60 years of age
  • Pathogenesis
    • autoantibodies (IgG) against desmoglein, a component of desmosome  
      • recall that desmosomes connect keratinocytes in the skin
      • type II hypersensitivity reaction
    • this disrupts keratinocyte adhesion and causes separation of epidermis
  • Associated conditions
    • drug use
      • thiols
        • penicillamine
        • benzylpenicillin
        • captopril
        • cephalosporin
    • thymoma
    • myasthenia gravis
    • systemic lupus erythematosus
  • Prognosis
    • without treatment, prognosis is poor
      • 75% mortality at 1 year
    • with treatment, prognosis is improved
      • 10% mortality at 1 year
Presentation
  • Symptoms
    • nonhealing and nonscarring ulcers persisting for at least 1 month
      • extremely painful
    • progressive development of blisters from mouth to the rest of the body
  • Physical exam
    • flaccid bullae that are easily ruptured
      • clear exudate
    • superficial erosions of skin (ruptured bullae)
      • often on scalp, face, and trunk
    • mucosal ulcers
    • positive Nikolsky sign
      • extension of blister or sloughing of skin with blunt pressure or lateral traction of skin
Studies
  • Labs
    • auto-antibodies detected  in serum with enzyme-linked immunosorbent assay (ELISA)
      • anti-desmoglein 3 (mucosal involvement)
      • anti-desmoglein 1 (mucocutaneous involvement)
  • Histology
    • acantholysis
      • separation of epidermal cells
    • intraepidermal blister
    • direct immunofluorescence
      • intercellular deposition of IgG or C3 in a net-like (reticular) pattern
Differential
  • Bullous pemphigoid
    • less severe than PV
    • does not affect mucous membranes
    • negative Nikolsky sign
Treatment
  • Conservative
    • wound care
      • indications
        • for all patients, to prevent secondary bacterial infection
      • antiseptics
      • anesthetic nebulizers for mucosal lesions
      • potent topical steroids
  • Medical
    • systemic steroids
      • indications
        • mainstay treatment
      • drugs
        • prednisolone
    • steroid-sparing immunosuppressants
      • indications
        • may be used concurrently with steroids for flares refractory to steroids
      • drugs
        • azathioprine
        • mycophenolate mofetil
Complications
  • Skin and soft tissue bacterial infection of open lesions
  • Risk of PV in newborn
    • due to placental transfer of maternal autoantibodies

 

 

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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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