Pruritus and pain management in EB

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Pruritus and pain management in EB

• Jemima Mellerio
• St Johns Institute of Dermatology
• Great Ormond Street Hospital
• London UK

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Pruritus and pain management in EB

• Pain Assessment
• Sources of pain in EB
• General measures
• Specific measures
• Pruritus General measures
• EB pruriginosa

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Pain in EB

• Pain is a feature for all people with EB
• Individual the same tissue damage may cause
  different level of pain in different people
• The same procedure may cause different levels of
  pain at different times in an individual

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Pain in EB assessment

• Individual and patient-focused
• Cultural and social influences
• Use a recognised tool e.g. visual analogue scale,
  Mc Gill pain questionnaire, pictorial

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Pain in EB assessment
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Pain in EB assessment
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Pain in EB assessment

• Neonates and infants
• restlessness
• persistent shrill cry
• raised heart and respiratory rate
• raised blood pressure
• drawn up limbs
• chin trembling

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Pain in EB sources
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• Blisters
• Wounds
• Esophageal - blisters and reflux
• Dental
• Corneal erosions
• Anal fissures
• Procedural pain e.g. dressings, bathing,
  venepuncture

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• Inflammation of skin and mucosae
• Neuropathic pain
• Constipation
• Contractures
• Osteoporosis

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Pain in EB general measures

• WHO analgesia ladder

Strong opioids /- non-opioids
Step 3
Weak opioids /- non-opioids
Step 2
NSAIDs Paracetamol
Step 1
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Pain in EB general measures

• If anxiety, consider midazolam
• May require long-acting opioid for chronic pain
  e.g. MST
• Fentanyl lozenges useful for procedural and
  break-through pain
• Neuropathic pain may respond to amitriptyline,
  nortriptyline, gabapentin or pregabalin

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Pain in EB general measures

• Non-pharmacological measures
• physiotherapy
• hydrotherapy
• guided imagery
• distraction
• acupuncture/acupressure

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Pain in EB specific measures

• Try to assess all types of pain
• May need several different approaches
  simultaneously
• Need to individualise pain management

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Pain in EB bullae and wounds

• Give analgesia before dressings
• Lance tense blisters
• Ethyl chloride spray immediately beforehand
• Have dressings ready
• Prepare the environment
• Distraction
• Atraumatic dressings

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Pain in EB bullae and wounds

• Topical opioids
• 10 mg morphine
• sulphate in 15 g
• hydrogel (Intrasite)
• with dressing
• changes
• (up to 0.2mg/kg morphine)

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Pain in EB oral mucosa

• Specialised teats for infants e.g. Haberman
  feeder
• Lubricate teats with teething gel
• Difflam spray
• NSAIDs in older children and adults
• Gelclair to coat oral mucosa

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Pain in EB esophagus

• Reflux can be a big problem
• Stepped approach with H2 blocker (ranitidine),
  domperidone and proton pump inhibitor (omeprazole
  or lansoprazole)
• Nebulised or systemic steroids if acute
  blistering
• Esophageal dilatation if necessary

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Pain in EB anus

• Perianal blisters or fissures
• Stool softeners e.g. Movicol (polyethylene
  glycol), increased fibre e.g soluble fibre in
  feeds, adequate fluid intake
• Lubricate anal margin before bowels open e.g. KY
  jelly, 5 lignocaine gel

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Pain in EB corneal erosion

• Regular ophthalmology review
• Lubricating eye drops and ointments especially if
  ectropion
• Treat blepharitis
• Regular analgesia for acute injury
• Avoid light
• Apply drops and ointments to lid margin if eye
  closed

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Pain in EB bones

• Osteoporosis common in EB especially severe types
• May be associated vertebral crush fractures
• Calcium and vitamin D supplements if necessary
• Bisphosphonates to relieve bony pain e.g. IV
  pamidronate, po risedronate

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Pain in EB malignancy

• Use stepped approach
• No fixed upper limit on opioids
• Different modalities surgery, radiotherapy,
  chemotherapy, systemic steroids
• Involvement of Hospice and Palliative Care Team
• Hollistic approach to management of terminal
  disease

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Pruritus in EB
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Pruritus in EB

• Very common
• Very distressing
• Affects all aspects of life including sleep
• Very resistant to treatment

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Pruritus in EB

• Emollients e.g. Aloe Propolis
• Dermol cream
• Menthol-containing
• Antihistamines disappointing but sedation may
  be useful
• Topical steroids disappointing and limits to use
• Psychological approaches e.g. distraction

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EB pruriginosa

• Clinical variant of DEB
• Differential includes lichen planus, nodular
  prurigo or artefact
• Recessive or dominant
• May co-exist with ordinary DEB in the family
• ? Etiology?IgE, atopy
• May be delayed onset

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EB pruriginosa

• Emollients
• Antihistamines
• Ondansetron
• Topical steroids
• Wet wraps
• Honey dressings and ointment
• Topical tacrolimus or pimecrolimus
• Cyclosporin
• Thalidomide

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Other causes of itch in EB

• Xerosis
• Eczema
• Contact and irritant dermatitis
• Drug rashes
• Scabies
• Urticaria and dermographism

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Pain management in EB summary
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Pruritus in EB summary
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References

• Fine JD et al. Assessment of mobility, activities
  and pain in different subtypes of epidermolysis
  bullosa. Clin Exp Dermatol 2004 29 122-7.
• Herod J et al. Epidermolysis bullosa in children
  pathophysiology, anaesthesia and pain management.
  Paediatr Anaesth 2002 12 388-97.
• Watterson G et al. Peripheral opioids in
  imflammatory pain. Arch Dis Childhood 2004 89
  679-81.