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