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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 .