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CLINICAL REPORT ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Pain Quality Assessment Scale for Epidermolysis Bullosa
Nicholas H. B. SCHRÄDER, Wing Y. YUEN and Marcel F. JONKMAN University of Groningen, University Medical Center Groningen, Center for Blistering Diseases, Department of Dermatology, Groningen, The Netherlands
Pain is one of the most debilitating symptoms in epidermolysis bullosa( EB) leading to reduced quality of life. Pain in EB comprises both neuropathic and nonneuropathic qualities. An assessment of pain qualities has not formerly been completed in EB. The Pain Quality Assessment Scale( PQAS) is an adjusted version of the validated Neuropathic Pain Scale and includes 20 pain qualities and descriptors. Patients with EB( n = 43) rated the pain qualities in the PQAS on 20 numerical scales and 1 multiple choice question. Pain was experienced by 39 patients( 91 %). In general, patients with EB experience intense and unpleasant pain on the surface of the skin; the hands and feet are most commonly affected. The subtypes, recessive dystrophic EB and junctional EB reported pain qualities pathognomonic of neuropathic pain. The PQAS adds value to the current practice of global pain intensity scoring in EB.
Key words: epidermolysis bullosa; pain; survey. Accepted Oct 19, 2017; Epub ahead of print Oct 23, 2017 Acta Derm Venereol 2018; 98: 346 – 349.
Corr: Marcel F. Jonkman, Department of Dermatology, University Medical Center Groningen, Hanzeplein 1, NL-9700 RB Groningen, The Netherlands. E-mail: m. f. jonkman @ derm. azg. nl
Epidermolysis bullosa( EB) is a group of inherited mucocutaneous blistering disorders that lead to painful erosions, strictures and contractures. The incidence of EB is 1:22,000 births and the prevalence is 2.4:100,000( 1); a total of 587 patients are currently registered in the Netherlands( population approximately 17 million). The disease is caused by mutations in structural proteins maintaining the integrity of stratified squamous epithelia. The splitting of skin and mucous membranes and subsequent blistering lead to a cascade of secondary effects that require comprehensive multidisciplinary clinical care, in which pain has been identified as one of the most significant and unbearable aspects( 2).
The most important issues in EB include the symptoms experienced by patients on a daily basis. One of the greatest challenges reported by patients is controlling pain during dressing changes( 3). Therefore, new research on effective pain alleviation, improving best-practice guidelines, will have an immediate and far-reaching effect on clinical practice.
EB research encompassing pain has been limited by a tendency to highlight global pain intensity rather than measure individual qualities of pain( 4). This is in contrast to current consensus that pain qualities or descriptors can aid the clinical classification of the nature of pain( e. g. neuropathic) and give direction to appropriate treat ments( 5). Not identifying the qualities of pain limits the ability to tailor treatment regimens for individual patients, as pharmacological treatments of neuropathic and non-neuropathic pain differ, and exposes patients to unnecessary health risks and healthcare costs for analgesic medicines( 6).
Pain in EB is highly associated with wound presentation, is extremely debilitating, and correlates strongly with a reduction in patients’ quality of life( QoL)( 3).
The general consensus is that pain in EB is caused by neuropathic and non-neuropathic mechanisms, probably exacerbated by central nervous system sensitization and psychological conditioning. Pain associated with extensive wounds has neuropathic qualities as it has a“ burning” sensation. A recent study confirmed a source of neuropathic pain as they showed a decreased intraepidermal nerve fibre density in patients with recessive dystrophic EB( RDEB), caused by injury( probably due to trauma, metabolic-toxins, infections and nutritional deficits, amongst others) to the distal terminals of small fibres( 7).
The most updated set of evidence-based best-practice EB guidelines addresses the many painful scenarios or events, including pain from surgery, chronic wounds, dressing changes, baths, ulcerative lesions, gastrointestinal tract, bones, corneal abrasions and end-of-life scenarios( 3). In the daily life of patients with EB, analgesia is key to coping with painful symptoms of multisystem disease. The use of allopathic drugs for every cause of pain may lead to a level of sedation that will hinder normal productivity and the ability to perform other daily activities. This poses a problem, as there is not one form of adequate pain therapy for these patients( 3).
The complex pathophysiology of EB means that the causes of pain cannot always be identified and treated appropriately. The aim of this study is to harness the clinical descriptors of pain qualities in EB by applying the Pain Quality Assessment Scale( PQAS), which will enable more accurate assessment of treatment and intervention outcomes through patient-reported pain qualities.
MATERIALS AND METHODS Study design
This study is a cross-sectional survey for patients diagnosed with any subtype of EB, registered at the Center for Blistering Diseases at the Department of Dermatology, University Medical Center Groningen( UMCG). doi: 10.2340 / 00015555-2827 Acta Derm Venereol 2018; 98: 346 – 349
This is an open access article under the CC BY-NC license. www. medicaljournals. se / acta Journal Compilation © 2018 Acta Dermato-Venereologica.