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Dermatological manifestations of malignancies and dermatological emergencies due to malignancy
Published in Biju Vasudevan, Rajesh Verma, Dermatological Emergencies, 2019
This is related to a myeloproliferative disorder with thrombocytosis in 20% of cases preceding malignancy. Erythromelalgia is defined by severe attacks of burning pain, erythema, and warmth of the distal extremities that can be relieved by cold exposure and/or elevation of the extremities. Since intravascular platelet activation and aggregation cause the distal arterioles to become occluded, antiplatelet agents like high-dose aspirin are useful.
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Published in Anton Sebastian, A Dictionary of the History of Medicine, 2018
Erythromelalgia Red neuralgia, a condition associated with painful feet. Mentioned by Robert Graves (1796–1853) in 1848. It was described in detail and named by American neurologist, Silas Weir Mitchell (1829–1914) of Philadelphia in 1872.
Practice Paper 6: Answers
Published in Anthony B. Starr, Hiruni Jayasena, David Capewell, Saran Shantikumar, Get ahead! Medicine, 2016
Anthony B. Starr, Hiruni Jayasena, David Capewell
Essential thrombocythaemia (ET) is a myeloproliferative disorder characterized by excessive production of defective platelets derived from abnormal megakaryocytes in the bone marrow. The main complications of ET are due to the formation of thrombi within the microcirculation. Symptoms include headache, digital ischaemia and abdominal pain secondary to splenic infarction. Many patients also suffer from erythromelalgia, i.e. aching and burning of the hands and feet secondary to ischaemia. Occasionally, there is paradoxical bleeding from the mucous membranes, such as the gastrointestinal tract and uterus. Investigation usually shows a platelet count in excess of 1000 × 109/L. The haemoglobin concentration and haematocrit are normal (unless there is significant blood loss, in which case there may be a microcytic anaemia). Definitive diagnosis requires bone marrow aspiration, which typically shows a hypercellular marrow with large numbers of megakaryocytes. Asymptomatic disease often requires no intervention. Severe and symptomatic disease can be treated with chemotherapy, usually with hydroxyurea or interferon-β, and low-dose aspirin.
Validation and reliability of current guidelines for the treatment of essential thrombocythemia under real-world clinical settings in Japan
Published in Hematology, 2022
Terumi Baba, Yoshinori Hashimoto, Hajime Yasuda, Marito Araki, Yoko Edahiro, Soji Morishita, Tomonori Ochiai, Shuichi Shirane, Jun Ando, Norio Komatsu
Major thrombotic events were defined as stroke, myocardial infarction, pulmonary embolism, and peripheral arterial occlusive disease. Minor thrombotic events included erythromelalgia, angina pectoris, transient ischemic attack, and deep vein thrombosis. Hemorrhagic events included cerebral hemorrhage, gastrointestinal hemorrhage, hematuria, and mucosal hemorrhage. Major hemorrhagic events were defined as cerebral or retroperitoneal bleeding, overt hemorrhage accompanied by a decrease in hemoglobin (Hb) ≥2 g/dl, or overt hemorrhage requiring blood transfusions of two units or more. Minor hemorrhagic events were defined as other hemorrhagic events not fulfilling the criteria of major hemorrhagic events [19,20]. Erythromelalgia was diagnosed according to the clinical diagnostic criteria proposed previously [21].
Simvastatin-induced erythromelalgia: less is more
Published in Acta Clinica Belgica, 2021
Yuran Vanwonterghem, Samyah Shadid
Based on this description, we concluded that the patient most likely suffered from erythromelalgia. This rare disorder (incidence of <2 per 100,000 people per year) is characterized by intermittent pain, heat and redness, usually in the lower extremities, but upper extremities and even the face have been described [1]. Typically, it is triggered by heat and exercise, but sometimes just by lowering the limbs, and improves only by cooling and elevating the affected limbs [1–3]. In general, classical pain killers provide little relief. Although the exact pathogenesis is poorly understood, it is thought to result from dysbalances in autonomic control of vascular tonus [3] which elicit hypoxia in the affected area, followed by a reactive hyperemia. This process may be associated with microvascular arteriovenous shunting, and result in local redness, warmth and burning pain [1,4]. Increased thrombocyte aggregation may play a role, but this has only been described in association with myeloproliferative diseases (see below) [4].
Clinical diagnosis and management of small fiber neuropathy: an update on best practice
Published in Expert Review of Neurotherapeutics, 2020
Grazia Devigili, Daniele Cazzato, Giuseppe Lauria
In SFN associated with sodium channel disorders, a selective block of peripheral sodium channel is supposed to improve sensory symptoms. Erythromelalgia has been successfully treated with mexiletine [124]. Most recently lacosamide, an anticonvulsant that acts on Nav1.3, Nav1.7, and Nav1.8, showed significant effect on pain, wellbeing, and sleep quality in SFN patients harboring Nav1.7 mutations [125]. One further study demonstrated that lacosamide can selectively enhance fast inactivation of the channel only in responders, unraveling the biophysical variability underpinning the responsiveness [126].