Best Clinical Practice Guidelines In Pain Management In Eb

Keneth Goldschneider, MD

Inherited Epidermolysis Bullosa (EB) comprises a group of rare disorders, generally thought of as skin diseases. However, EB has multi-system effects and patients present with a number of both acute and chronic pain care needs. Effects on quality of life are substantial. Due to its low prevalence, expertise in pain care for patients with this disease is often restricted to a few specialized care centres. Even then, evidence-based pain care is limited by a near absence of scientific literature specific to EB. This set of guidelines was requested by DEBRA International, to help standardize the approach to pain care for patients with EB in all parts of the world. Consequently, a group of clinical pain care experts from a few countries have come together to lend their experience to the limited scientific literature to create these guidelines. In order to generate useful clinical guidelines, an approach was taken that combined extrapolation from related literature and expert consensus.


The present guidelines on pain care for patients with EB are based on a review and synthesis of the available literature, guided by expert consensus and thoughtful application of theory. The guidelines are divided into three topics: acute pain, chronic and recurrent pain, and special topics. The acute pain section focuses on postoperative pain. The chronic and recurrent pain section includes dressing changes, baths, skin pain and joint and other body pains. Special topics include pain care in infants with EB, psychological approaches to pain care, and palliative pain care. While not pain per se, itching is a major source of discomfort and is also discussed. For each topic, research questions are presented to act as prompts for thinking about the next steps in producing evidence to support updated guidelines.

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