Rosen & Barkin's 5-Minute Emergency Medicine Consult (520 page)

DIAGNOSIS

• Generalized or focal flaccid bullae (blisters) of the skin and mucosa
  
• Painful skin erosions with shreds of detached epithelium
  
• Painful nonhealing oral, vaginal, or mucosal erosions
  
• Crusting, partially healing skin erosions from ruptured bullae
  
• Hypertrophic, hyperplastic erosive plaques with pustules in intertriginous areas (pemphigus vegetans)
  
• Moist, edematous, exfoliative erosions in seborrheic areas (pemphigus foliaceus)
  
• Erythematous, scaly, crusting skin lesions in a malar distribution (pemphigus erythematosus)
  
• Lesions usually persist without treatment:
  
  • May heal with post inflammatory hyperpigmentation
    

• Typically features mucocutaneous blisters followed by erosions
  
• Often appear 1st in mucous membranes with spread to cutaneous involvement; most commonly to scalp, chest, axillae, and groin
  
• Skin lesions are painful flaccid blisters that may appear anywhere
  

Nikolsky sign (separation of the epidermis with lateral pressure) is characteristic but not diagnostic:

• Poor sensitivity
  

• Suspected based on clinical presentation
  
• Biopsy with histologic and immunofluorescence testing is essential for definitive diagnosis (arrange with a dermatologist)
  

• Serum antibody titers, detected by indirect immunofluorescence, are often used as a marker of disease activity; however, the ED physician usually does not order these titers
  
• ELISA may be used to identify subtypes
  

No diagnostic imaging test exists

Deep shave or punch biopsy

• Bullous pemphigoid
  
• Contact dermatitis
  
• Dermatitis herpetiformis
  
• Erythema multiforme
  
• Erysipelas
  
• Erythroderma
  
• Toxic epidermal necrolysis
  
• Epidermolysis bullosa
  
• Hand, foot, and mouth disease
  
• Systemic lupus erythematosus
  
• Systemic vasculitis
  
• Oral candidiasis
  
• Herpes simplex gingivostomatitis
  
• Erosive lichen planus
  
• Seborrheic dermatitis
  

TREATMENT

• If severe disease:
  
  • IV access, pulse oximetry monitor, and cardiac monitor
    

• If symptoms of hypotension or sepsis are present, IV fluid resuscitation should be guided by the Parkland burn formula
  
• If signs or symptoms of sepsis are present, initiate broad-spectrum antibiotic coverage
  
• In steroid-dependent patients, administer stress-dose steroids
  

• Systemic corticosteroids are the mainstay of therapy
  
• Mild-to-moderate disease
  should receive PO prednisone, and intralesional triamcinolone acetonide may be used
  
• Severe disease:
  Conventional high-dose corticosteroids:
  
  • If severe symptoms are unresponsive to high-dose PO corticosteroids, consider pulse IV corticosteroids and admission for plasmapheresis
    
• Adjuvant immunosuppressive therapy may also be added to decrease the symptoms associated with high-dose systemic corticosteroids or in patients with contraindications to steroid therapy:
  
  • Dapsone, gold, azathioprine, cyclophosphamide, cyclosporine, methotrexate, mycophenolate, and IV immunoglobulins
    

• Immune suppression:
  
  • Hydrocortisone: 100–300 mg/d IV stress-dose steroids adjusted based on patients known dosage and use habits
    
  • Methylprednisolone (pulse IV therapy; adults): 1 g IV over 3 hr daily
    
  • Prednisone: 1 mg/kg/d PO daily (adults); moderate-to-severe disease PO daily for 5–10 wk, then taper
    
  • Triamcinolone acetonide for limited intraoral involvement – 10 mg/mL 0.1-mL injection into each superficial lesion
    
• Pain:
  
  • Opiates, anti-inflammatory agents, acetaminophen
    
  • Biobrane synthetic dressing
    
  • Diphenhydramine and Maalox or Xylocaine oral wash
    

• Usually performed as an inpatient for severe, refractory cases
  
• Immune suppression:
  
  • IVIG: single cycle 400 mg/kg per day for
    
  • 5 days
    
  • Rituximab
    
  • Triamcinolone acetonide: 10 mg/mL 0.1-mL injection into each superficial lesion
    
• Pain:
  
  • Gabapentin 300 mg daily titrated up to 300 mg TID over a month
    
• Other considerations: Patients on high-dose steroids should have diets high in vitamin D and calcium and may benefit from a proton-pump inhibitor or bisphosphonates.
  

FOLLOW-UP

• Most acute flares are minor and can be managed with PO glucocorticoids and dermatology follow-up
  
• Admit 1st-time presentations of disease to facilitate treatment and definitive diagnosis with biopsy and rule out of high morbidity blistering skin disease
  
• Admit patients with extensive mucocutaneous involvement, intractable pain, coexisting bacterial skin infection, or signs of sepsis
  
• Admit to a floor bed if pulse parenteral steroid therapy or plasmapheresis is indicated
  
• Admit to the ICU or burn unit if any signs and symptoms of shock or sepsis are present because aggressive fluid resuscitation, wound care, and multiple medications will be required
  

• Discharge if mild-to-moderate disease will not require aggressive steroid management, plasmapheresis, or aggressive pain control
  

• A follow-up evaluation with dermatology is essential to monitor the course of the disease and to adjust treatment
  
• Rheumatology follow-up may be advantageous to assess risk of osteoporosis via bone scan if on high-dose steroids
  

• Mucocutaneous lesions often begin on face, head/scalp, or oral cavity
  
• Long-term management is the rule; ensure proper dermatology follow-up
  
• Glucocorticoids are the mainstay of therapy
  
• Paraneoplastic type often with severe oral mucosal involvement, consider associated lymphoproliferative disorder
  
• Patients on immunosuppressive treatment including steroids and immunomodulating agents are at very high risk of complications and may present in adrenal crisis, severe sepsis, or hyperosmolar nonketotic acidosis secondary to new-onset type 2 diabetes
  
• Patients with hypotension require aggressive fluid resuscitation
  

• Amagai M, Ikeda S, Shimizu H, et al. A randomized double-blind trial of intravenous immunoglobulin for pemphigus.
  J Am Acad Dermatol
  . 2009;60:595–603.
  
• Kasperkiewicz M, Schmidt E, Zillikens D. Current therapy of the pemphigus group.
  Clin Dermatol.
  2012;30:84–94.
  
• Kavusi S, Daneshpazhooh M, Farahani F, et al. Outcome of pemphigus vulgaris.
  J Eur Acad Dermatol Venereol.
  2008;22:580–584.
  
• Langan SM, Smeeth L, Hubbard R, et al. Bullous pemphigoid and pemphigus vulgaris – incidence and mortality in the UK: Population based cohort study.
  BMJ
  . 2008;337:a180.
  
• Martin LK, Werth VP, Villaneuva EV, et al. A systematic review of randomized controlled trials for pemphigus vulgaris and pemphigus foliaceus.
  J Am Acad Dermatol
  . 2011;64:903–908.
  
• Rashid RM, Candido KD. Pemphigus pain: A review on management.
  Clin J Pain
  . 2008;24:734–735.
  
• Schmidt E, Waschke J. Apoptosis in pemphigus.
  Autoimmun Rev
  . 2009;8:533–537.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Erythema Multiforme
  
• Rash
  
• Toxic Epidermal Necrolysis
  

CODES

• 684 Impetigo
  
• 694.4 Pemphigus
  
• 694.6 Benign mucous membrane pemphigoid