CLINICAL REPORT
185 ActaDV ActaDV Advances in dermatology and venereology Acta Dermato-Venereologica
Burden of Aquagenic Pruritus in Polycythaemia Vera
Edyta LELONEK 1 , Łukasz MATUSIAK 1 , Tomasz WRÓBEL 2 , Jacek KWIATKOWSKI 2 and Jacek C . SZEPIETOWSKI 1
1
Department and Clinic of Dermatology , Venereology and Allergology , and 2 Department and Clinic of Hematology , Blood Neoplasms and Bone Marrow Transplantation , Wroclaw Medical University , Wrocław , Poland
Aquagenic pruritus ( AP ) has a significant influence on quality of life ( QoL ) in patients with polycythaemia vera . This study analysed the impact of AP on patient well-being in 102 patients with polycythaemia vera . Intensity of pruritus was evaluated using a visual analogue scale ( VAS ), verbal rating scale ( VRS ) and a 4-item Itch Questionnaire . Psychosocial aspects of AP were assessed with the Hospital Anxiety and Depression Scale ( HADS ), EQ-5D and itch-specific QoL questionnaire ( ItchyQoL ). AP of mean duration 6.6 ± 8.6 years and intensity 4.8 ± 1.9 points ( VAS ) was present in 42 / 102 individuals . The prevalence of depression and anxiety among patients with AP was 23.8 % and 9.5 %, respectively . Depression was more frequent in the AP group ( vs . non-AP ). Moreover , patients with AP had higher HADSanxiety scoring than those without pruritus ( p = 0.005 ). A negative correlation was found between duration of AP and EQ-5D-VAS . The ItchyQol score of 37.3 ± 12.3 points was influenced by the extent ( p = 0.01 ) and duration of episodes of AP ( p = 0.02 ). In conclusion , AP places an additional burden on patients with polycythaemia vera , negatively influencing their QoL .
Key words : aquagenic pruritus ; polycythaemia vera ; hydrophobia ; anxiety ; depression .
Accepted Oct 2 , 2017 ; Epub ahead of print Oct 3 , 2017 Acta Derm Venereol 2018 ; 98 : 185 – 190 .
Corr : Jacek C . Szepietowski , Department and Clinic of Dermatology , Venereology and Allergology , Wroclaw Medical University , Chałubińskiego 1 , PL-50-368 Wrocław , Poland . E-mail : jacek . szepietowski @ umed . wroc . pl
Aquagenic pruritus ( AP ) is a debilitating dermal condition characterized by the development of intense itching , stinging , tingling or burning sensations , without observable skin lesions , brought on by contact with water of any temperature ( 1 ). AP is recognized to be a pathognomonic sign of polycythaemia vera ( PV ), which is one of the main clonal haematological malignant neoplasms caused by mutations of a cytoplasmic tyrosine kinase , Janus kinase 2 enzyme ( either JAK2V617F or JAK2 exon 12 ) ( 2 , 3 ). Furthermore , homozygotic mutations of JAK2 among patients with PV were found to be significantly more often associated with AP ( 4 ).
PV-associated pruritus was first reported in 1985 ( 1 ) and its estimated prevalence varies , according to data , from 31 % to 69 % of patients ( 5 – 7 ). Notably , the onset of AP precedes diagnosis of PV in almost half of cases ( 8 ).
AP has a significant influence on patients ’ quality of life ( QoL ), depriving patients of sleep , and can lead to development of a psychological phobia and abandonment of bathing ( 9 ). Moreover , the majority of patients believe that pruritus is the most unmanageable aspect of PV , which may lead , in more severe cases , to a reduced physical and social life ( 1 ).
Although AP is recognized as the most excruciating aspect of PV , studies evaluating its impact on the psychosocial state of patients with AP are limited ( 1 , 8 ). The aim of this study was therefore to analyse the clinical features of AP , and its influence on patient well-being , in a group of patients with PV .
MATERIALS AND METHODS Patients
The study was conducted on a group of 102 patients ( 65 females , 37 males ), with PV diagnosed according to the WHO criteria . All of the patients had the JAK2V617F mutation . The age range of the studied individuals was 30 – 90 years ( mean ± standard deviation ( SD ) 66.9 ± 12.7 years ). The majority of PV patients were treated with 5-hydroxyurea ( 5-HU ) ( 71.6 %) and phlebotomy ( 66.7 %). Less frequently , patients were treated with acetylsalicylic acid ( 40.2 %), clopidogrel ( 9.8 %), anagrelide ( 7.8 %) and pipobroman ( 2.9 %). PV was adequately controlled in 45.6 % of patients ( 10 ). All patients underwent a physical examination and , those who reported pruritus brought on by contact with water with no observable skin lesions , were treated as having AP . The individuals who had pruritus of origin other than AP ( e . g . senile pruritus , atopic itch , etc .) were excluded from the AP group . Only 3 patients had anti-pruritic treatment with antihistamines . The detailed characteristics of the study group are shown in Table I . Patients without AP served as matched controls ( with regard to age , sex , body mass index ( BMI ), duration of PV and number of thrombotic episodes ).
The study was approved by the Ethics Committee of Wroclaw Medical University ( No . 355 / 2016 ).
Study design
Demographic data , disease history , PV status and treatment modalities were collected from all participants . Unidimensional scales were used to evaluate AP intensity , including a visual analogue scale ( VAS ) and verbal rating scale ( VRS ). With regard to VAS , the patients were asked to report , and mark on the 10-point scale , the maximum and mean intensity of pruritus they had experienced during the last 3 days . The scores ranged from 0 ( no itch ) to 10 points ( worst imaginable itch ). VAS scoring is as follows , mild pruritus ( 0 –< 3 points ), moderate pruritus ( ≥ 3 – 7 points ), severe pruritus ( ≥7 – 9 points ) and very severe pruritus ( ≥ 9 points ).
In VRS , participants assessed their itch intensity as : “ mild ”, “ moderate ”, “ severe ” or “ very severe ”. In addition , the pruritus was characterized with a 4-item Itch Questionnaire . This instrument was successfully utilized previously by our group to estimate the extent ( 1 – 3 points ), intensity ( 1 – 5 points ), frequency ( 1 – 5 points ) and sleep disturbances ( 0 – 6 points ) caused by pruritus of various
This is an open access article under the CC BY-NC license . www . medicaljournals . se / acta Journal Compilation © 2018 Acta Dermato-Venereologica . doi : 10.2340 / 00015555-2812 Acta Derm Venereol 2018 ; 98 : 185 – 190