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Pruritus induced or exacerbated by exposure to water — aquagenic pruritus (AP) — is a well-known symptom that accompanies polycythemia vera (PV), formerly known as polycythemia rubra vera. Patients usually describe itching, but other skin symptoms may occur as well. Little information is available regarding prevalence, effects on life quality, and proper management of AP in patients with PV.
A large cohort of PV patients was surveyed. Of 441 patients, 301 had AP. Most described itching (71.8%), but others reported tingling, burning, or stinging. The pruritus occurred mostly on the trunk and proximal extremities. In 64.8% of AP-affected patients, AP symptoms were present an average of 2.9 years before PV was diagnosed. AP symptoms prompted PV investigation in only 15.4% of these patients. AP symptoms were described as “unbearable” by 14.8%. AP-directed therapy was prescribed for only a quarter of affected patients; this was, primarily, antihistaminic agents, but small numbers received antidepressants, gabapentin, or ultraviolet B phototherapy. AP symptom response to various PV-directed therapies was very variable. Quality of life was poorer in patients with AP than in those without.
Siegel FP et al. Aquagenic pruritus in polycythemia vera: Characteristics and influence on quality of life in 441 patients. Am J Hematol 2013 Aug; 88:665. (http://dx.doi.org/10.1002/ajh.23474)
Comment
This important paper details the experience of a large number of patients from the patients' perspective. Recall bias might have affected the findings of this survey tool, but there are still a number of clinically significant findings. It is striking that only a small number of patients who described aquagenic pruritus were investigated for polycythemia vera, but whether these patients consulted generalists or specialists, including dermatologists, is unknown. The findings should remind us to obtain a complete blood count in patients presenting with AP. This report opens the door to assessment of therapies, directed at PV or at the pruritus, that improve patient quality of life. The authors suggest the advent of JAK2 inhibitors may bring progress.