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Systemic Diseases and the Skin
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Jana Kazandjieva, Razvigor Darlenski, Nikolai Tsankov
Polycythemia vera is an example of a chronic myeloproliferative disorder of myeloid cells that results in an increased red cell mass. The vascular complications in patients with polycythemia vera include a plethora and flushing of the face and palms, aquagenic pruritus associated with a hot shower in 40% of patients), erythromelalgia, livedo reticularis, acrocyanosis, and pyoderma gangrenosum.
Management of Polycythemia Vera
Published in Richard T. Silver, Ayalew Tefferi, Myeloproliferative Disorders, 2007
Shireen Sirhan, Richard T. Silver
One of the most troubling and poorly explained symptoms associated with PV is aquagenic pruritus, which occurs in some patients during the course of their disease (40). This particular symptom can sometimes become severe enough to prevent patients from taking daily showers and also interferes with their social activities. Interferon (rIFN-a) is the only medication, which will reliably reduce the intensity of and relieve the symptoms in most cases. Leukemic transformation in PV is a dreaded complication with no effective therapy (41). The incidence is estimated at approximately 5–10% in the first 10 years of the disease and at least twice as much in the second decade of the disease (42). This particular complication is primarily attributed to the intrinsic biology of the disease and is further modified by modern therapeutic agents. The relative contribution is open to debate and will be elaborated in a later section.
Clinical management
Published in Alistair Burns, Michael A Horan, John E Clague, Gillian McLean, Geriatric Medicine for Old-Age Psychiatrists, 2005
Alistair Burns, Michael A Horan, John E Clague, Gillian McLean
Itch in normal-appearing skin may be a feature of systemic disease. In the elderly especially, itch may occur in normal-appearing skin in the absence of systemic disease. In this setting the itching is thought to be due to reduced water content of the skin without evident dryness. Itching with- out skin changes is also seen in that caused by water - aquagenic pruritus. Common skin rashes affecting older people are described on page 181.
Current approaches to challenging scenarios in myeloproliferative neoplasms
Published in Expert Review of Anticancer Therapy, 2018
Eran Zimran, Ronald Hoffman, Marina Kremyanskaya
MPN-associated pruritus is described by patients as an itching, tingling, or burning sensation typically distributed on the upper trunk or proximal limbs. Itching can occur spontaneously, or appear after specific environmental triggers such as contact with water (aquagenic pruritus), especially warm water. Additional triggers can be a sudden change of temperature, sweating, alcohol consumption, or use of warm bedclothes at night. Physical exam usually does not reveal skin lesions aside from signs of scratching [75]. Often patients describe pruritus as their most troublesome and disabling symptom. In fact, some patients report having to stop taking showers and resort to sponge bathing only. The presence of pruritus was found to be associated with reduced participation in physical and social activities, emotional liability, sleeping disturbances and depression [72].
A spotlight on the management of complications associated with myeloproliferative neoplasms: a clinician’s perspective
Published in Expert Review of Hematology, 2018
Graeme Greenfield, Mary F. McMullin
Pruritus is a prevalent symptom in many MPNs. It can be extremely disabling, interfering with sleep and has led to suicidal intent in some cases. Severe aquagenic pruritus can be a major factor which interferes with many activities of daily living. This, almost pathognomonic symptom of MPN, has a poorly understood etiology. Increased histamine and its release has long been thought to be associated [12]. Basophils have been found to be increased, hypersensitive, constitutively activated associated particularly in those with a JAK2 clone as part of an activated JAK/STAT pathway [13]. Histamine release and the activated basophils are thought to contribute to the etiology of the pruritus.
Improving symptom burden and quality of life in patients with myelofibrosis: current strategies and future directions
Published in Expert Review of Hematology, 2021
Pruritus, particularly after exposure to warm water (aquagenic pruritus), is one of the most common symptoms reported by patients with PV [11,69]. Pruritus is relatively common in those with MF as well, with series reporting anywhere from 16% to 50% incidence in this setting [26,70]. Aquagenic pruritus is described by patients as an intense itching, tingling, stinging, or burning sensation triggered by contact with water, especially warm water, without visible skin lesions [69]. Aquagenic pruritus can be debilitating from both a physical and a psychosocial perspective; 15% of patients with PV rate it as ‘unbearable,’ and in extreme cases can prohibit bathing entirely [69].