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PubMed1997

Aspirin in essential thrombocythemia: status quo and quo vadis.

Aspirin has a well established role in the prevention of arterial thrombosis. Discussion on the efficacy and safety of aspirin in the treatment and prophylaxis of thrombosis in essential thrombocythemia (ET) has become an important issue. The rationale for its use in ET comes from the observation that arterial thrombosis and platelet-mediated microcirculatory disturbances are the major causes of morbidity and mortality in ET. Experimental data have shown persistently elevated levels of thromboxane A2 (TXA2) in ET patients probably reflecting an enhanced in vivo platelet activation. Increased TXA2 biosynthesis and platelet activation in vivo in ET are selectively suppressed by repeated low doses of aspirin. ET-related symptoms such as erythromelalgia, transient neurologic and ocular disturbances are sensitive to aspirin. However, the benefit of low-dose aspirin is still uncertain in the primary prevention of thrombosis in ET. Furthermore, aspirin may unmask a latent bleeding diathesis frequently present in ET which may result in severe hemorrhagic complications. Thus, aspirin is contraindicated in ET patients with a bleeding history or a very high platelet count (> 1500 x 10(9)/L) leading to the acquisition of von Willebrand factor deficiency. If indicated, aspirin is presently used in the widely accepted low-dose regimen of 100 mg daily. However, an optimal effective dose has not yet been established. To further evaluate the efficacy and safety of aspirin in ET, prospective clinical trials are needed.

Abortion, Habitual↗
PubMed2006

Platelet-mediated erythromelalgic, cerebral, ocular and coronary microvascular ischemic and thrombotic manifestations in patients with essential thrombocythemia and polycythemia vera: a distinct aspirin-responsive and coumadin-resistant arterial thrombophilia.

Microvascular circulation disturbances including erythromelalgia, its microvascular ischemic complications, and migraine-like atypical or typical transient ischemic cerebral, ocular, and coronary ischemic attacks are specific clinical manifestations in patients with essential thrombocythemia (ET), and polycythemia vera (PV) associated with thrombocythemia. Thrombocythemia (ET and PV) patients with microvascular disturbances have shortened platelet survival, increased beta-thromboglobulin (beta-tg), platelet factor 4 (PF4), and thrombomoduline (TM) levels, and increased urinary thromboxane B2 (TxB2) excretion indicating platelet-mediated processes in vivo. Inhibition of platelet cyclooxygenase (COX 1) by aspirin is followed by relief of microvascular disturbances, correction of shortened platelet survival, and return of plasma levels of beta-tg, PF4, TM levels and TxB2 excretion to normal. The transient ischemic attacks and thrombotic complications in thrombocythemia are very likely caused by hypersensitive platelets produced by spontaneously proliferating enlarged megakaryocytes in the bone marrow of ET and PV patients. In contrast to normal platelets in healthy individuals the circulating hypersensitive thrombocythemic platelets spontaneously activate and secrete their products, thus forming aggregates that transiently plug the microcirculation, or result in occlusive platelet thrombi in arterioles or small arteries. Clear evidence is presented that the microvascular transient ischemic and occlusive thrombotic complications in thrombocythemia patients are relieved by treatment with aspirin and by reduction of platelet counts to normal (<400 x 109/l), but not by coumadin. In patients with thrombocythemia associated with PV, increased hematocrit and whole blood viscosity aggravate the platelet-mediated microvascular ischemic and thrombotic syndrome of thrombocythemia to major arterial and venous thrombotic complications. Correction of hematocrit and blood viscosity by phlebotomy significantly reduces the major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated microvascular circulation disturbances in PV patients because thrombocythemia persists. Complete relief and prevention of microvascular and major thrombosis in PV patients are obtained by treatment with low-dose aspirin on top of phlebotomy or by treatment with the platelet lowering agents, anagrelide, interferon or hydroxyurea.

Aged↗
PubMed1996

Cutaneous pathology in primary erythermalgia.

Primary or idiopathic erythermalgia is characterized by recurrent, red, warm, and painful lower extremities. It arises at young age and persists throughout life because no treatment is available. We report the cutaneous pathology of affected skin lesions of three patients with primary erythermalgia. Biopsy specimens showed a mild perivascular mononuclear infiltrate, thickened blood vessel basement membranes, abundant perivascular edema, and moderate endothelial swelling. The thickened basal membrane of the blood vessels showed a laminar structure, and abundant perivascular edema and moderate endothelial cell swelling were evident. These histopathologic findings in primary erythermalgia appear to be nonspecific but allow diagnostic differentiation from erythromelalgia in which fibromuscular intimal proliferation and occlusive thrombi in the endarteriolar capillaries are apparent and from erythermalgia secondary to vasculitis. Histopathologic examination of affected skin lesions in patients with red, congested, warm, and painful burning extremities is a valuable tool in the diagnostic process.

Adolescent↗
PubMed1984

William Harvey and his gout.

In William Harvey's day almost any or every arthropathy was termed gout. This is evident in the case histories of some of his patients and in his own case, where his own cold water therapy would suggest the correct diagnosis was not gout but erythromelalgia (Weir Mitchell's disease).

Arthritis↗
PubMed2006

Inherited erythermalgia: limb pain from an S4 charge-neutral Na channelopathy.

BACKGROUND: Inherited erythermalgia (also termed "erythromelalgia"), characterized by episodic burning pain in the distal extremities evoked by warmth, has been causally linked with mutations of the Na(v)1.7 sodium channel, which is preferentially expressed in nociceptors. Thus far, Na(v)1.7 mutations within intracellular linker parts of the channel have been physiologically characterized. OBJECTIVE: To investigate a Na(v)1.7 erythermalgia mutation that substitutes one uncharged amino acid for another within an S4 segment. METHODS: Whole-cell patch-clamp analysis was used to study biophysical properties of wild-type and mutant (F216S) Na(v)1.7 channels in mammalian cells. RESULTS: The F216S mutation hyperpolarizes the voltage dependence of activation by 11 mV, accelerates activation, slows deactivation, and enhances the response to slow, small depolarizations. CONCLUSION: These results provide a physiologic basis for the linkage to erythermalgia of an Na(v)1.7 mutation that substitutes one uncharged residue for another within an S4 segment of the channel. These changes should increase excitability of nociceptive dorsal root ganglion neurons in which the mutant channel is present, thus contributing to pain.

Action Potentials↗
PubMed2006

New syndromes in mushroom poisoning.

Several new mushroom poisoning syndromes have been described since the early 1990s. In these syndromes, the onset of symptoms generally occurs >6 hours after ingestion. Treatment is mainly supportive. The syndrome induced by Amanita smithiana/proxima consists of acute tubulopathy, which appears earlier and does not have the same poor prognosis as the orellanine-induced syndrome. It has been described since 1992 in the US and Canada with A. smithiana; in France, Spain and Italy with A. proxima; and in Japan with A. pseudoporphyria. The responsible toxin is probably 2-amino-4,5-hexadienoic acid. The erythromelalgia syndrome has been described as early as the late 19th century in Japan and South Korea with Clitocybe acromelalga, and since 1996 in France and then Italy with C. amoenolens. Responsible toxins are probably acromelic acids identified in both species. Several cases of massive rhabdomyolysis have been reported since 1993 in France and 2001 in Poland after ingestion of large amounts of an edible and, until then, valuable species called Tricholoma equestre. These cases of rhabdomyolysis are associated with respiratory and cardiac (myocarditis) complications leading to death. Rhabdomyolysis with an apparently different mechanism was described in Taiwan in 2001 with Russula subnigricans. Finally, cases of encephalopathy were observed twice after ingestion of Hapalopilus rutilans in Germany in 1992 and Pleurocybella porrigens in Japan in 2004, where a convulsive encephalopathy outbreak was reported in patients with history of chronic renal failure.

Erythromelalgia↗
PubMed1996

Atypical transient ischemic attacks in thrombocythemia of various myeloproliferative disorders.

Neurological symptoms of transient unsteadiness, dysarthria, dysphasia, dysbasia, transient monoor hemiparesis, hemiparesis, scintillating scotomas, amaurosis fugax, vertigo, dizziness, migraine accompaniments, syncope and seizures were the presenting manifestations of thrombocythemia in various myeloproliferative disorders. Erythromelalgia preceded or followed the neurologic ischemic attacks. The neurologic and ocular attacks usually had a sudden onset, lasted for a few seconds to several minutes and occurred independently or sequentially rather than simultaneously. This clinical syndrome is caused by platelet-mediated ischemic and thrombotic processes in the end-arterial microvasculature and reflects the existence of a platelet dependent and aspirin responsive arterial thrombophilia in thrombocythemia as novel disease entity, which confirms and elucidates Mitchell's hypothesis.

Adult↗
PubMed2003

Skin blood flow in adult human thermoregulation: how it works, when it does not, and why.

The thermoregulatory control of human skin blood flow is vital to the maintenance of normal body temperatures during challenges to thermal homeostasis. Sympathetic neural control of skin blood flow includes the noradrenergic vasoconstrictor system and a sympathetic active vasodilator system, the latter of which is responsible for 80% to 90% of the substantial cutaneous vasodilation that occurs with whole body heat stress. With body heating, the magnitude of skin vasodilation is striking: skin blood flow can reach 6 to 8 L/min during hyperthermia. Cutaneous sympathetic vasoconstrictor and vasodilator systems also participate in baroreflex control of blood pressure; this is particularly important during heat stress, when such a large percentage of cardiac output is directed to the skin. Local thermal control of cutaneous blood vessels also contributes importantly--local warming of the skin can cause maximal vasodilation in healthy humans and includes roles for both local sensory nerves and nitric oxide. Local cooling of the skin can decrease skin blood flow to minimal levels. During menopause, changes in reproductive hormone levels substantially alter thermoregulatory control of skin blood flow. This altered control might contribute to the occurrence of hot flashes. In type 2 diabetes mellitus, the ability of skin blood vessels to dilate is impaired. This impaired vasodilation likely contributes to the increased risk of heat illness in this patient population during exposure to elevated ambient temperatures. Raynaud phenomenon and erythromelalgia represent cutaneous microvascular disorders whose pathophysiology appears to relate to disorders of local and/or reflex thermoregulatory control of the skin circulation.

Adult↗
PubMed2000

[What vascular events suggest a myeloproliferative disorder?].

Patients with polycethemia vera (PV) or essential thrombocythemia (ET) are at increased risk of arterial and venous thromboembolic events. Arterial ischemic complications occur in 24 to 43% of these patients, particularly those with cardiovascular risk factors (especially cigarette smoking). Non-atheromatous arterial thrombosis concerns all large and medium-sized vessels, particularly cerebral, limb, coronary and digestive arteries. Extensive complications have been described in patients with lower limb occlusive arteriopathy, particularly stent or bypass thrombosis, critical ischemia. Juvenile myocardial infarction or rapid postangioplasty coronary thrombosis may reveal certain myeloproliferative disorders, particularly ET. Venous thrombosis is more frequent in PV than in ET; superficial or deep venous thromboses are seen. Thromboembolic events occur in about 25-30% of the patients and account for one-third of the deaths. Mesenteric vein thrombosis, portal thrombosis, or suprahepatic vein thrombosis may occur in all myeloproliferative disorders, but the pathogenesis is not fully understood. Pulmonary hypertension may be the consequence of local thrombosis in the pulmonary vasculature or may be due to the high blood flow in the right heart cavities. Microvascular circulatory disturbance includes erythromelalgia, Raynaud's phenomenon, digital ischemia, acrocyanosis, blue toe syndrome, livedo reticularis, cutaneous ulcers or necrotic purpura. All these manifestations may precede the myeloproliferative disorder by several months.

Adult↗
PubMedDate not supplied

[GABApentin--new therapeutic possibilities].

The paper is a review of current experience with use of gabapentin--a new antiepileptic drug--in neurologic conditions others than epilepsy. Mechanism of action of the drug is not fully elucidated yet. However it proved to be effective in therapy of chronic pain, especially in neuropathic pain, neuralgia, low back pain, reflex sympathetic dystrophy and erythromelalgia. Gabapentin is also effective in pain and spasticity in multiple sclerosis. Clinical studies of gabapentin in movement disorders, such as Parkinson disease, essential tremor and atrophic lateral sclerosis are discussed in the paper. It can be summarized that gabapentin is a valuable medication and the use thereof in neurology is not limited to epilepsy.

Acetates↗
PubMed1990

["Acro-erythro-cyanosis"--peculiar vasomotor symptoms due to cervical hernial myelopathy].

Two cases of cervical myelopathy, which exhibited peculiar vasomotor symptoms ("acro-erythro-cyanosis") on distal regions of the four limbs are reported. Continuous reddening, swelling and skin temperature increase were observed on both hands and feet in case 1, a-44-year-old man, and on both hands in case 2, a-47-year-old man. Cold stimulation resulted in cyanosis and decrease of skin temperature on the affected regions paroxysmally. The condition of skin at room temperature may be caused by arteriectasia of arterioles due to hypotonia of vasomotor fibers, and this was similar to erythromelalgia. On the other hand, cold stimulation may have led to this condition, where the contraction of skin arterioles due to hypertonia of vasomotor fibers was added to the dilation of venule, and this was similar to acrocyanosis. Consequently, we provisionally named the vasomotor symptoms in the present cases as "acro-erythro-cyanosis". The other neurological signs were as follows. Case 1: mild weakness in right upper and lower limbs and left small hand muscles, mild superficial and deep sensory disturbance on bilateral palms and soles and decrease of vibration on bilateral lumbar regions and thereunder. Case 2: mild weakness of right small hand muscles, superficial sensory disturbance on distal regions of bilateral upper and lower limbs and a decrease of joint position sense on right hand. Myelography and metrizamide CT myelography revealed a high-degree deformity of the spinal cord due to the herniated disks between C4 and C5 in case 1 and between C3 and C4 in case 2.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1989

[The acrodynic form of the Riley-Day syndrome].

The authors report the case of a 10-year-old girl who died after suffering from severe erythromelalgia combined with digestive and general disorders associated with multiple vegetative disturbances (high blood pressure, hypothermia, colic and urinary disorders, raised catecholamine levels) for 2 years. Although the appearance of the tongue was normal and in spite of the absence of genetic criteria the diagnosis of Riley-Day dysautonomia was made and its association with neural crest disorders was indicated.

Child↗
PubMed1988

[Erythermalgia].

Erythromelalgia is a vascular acrosyndrome affecting mainly the feet and triggered by elevation of room temperature. It may be primary, or secondary, in particular to a polycythemia, when an increased platelet count plays an essential role, explaining the beneficial effect of aspirin or NSAI agents that inhibit platelet prostaglandin cyclo-oxygenase. The favorable action of beta-blockers could result from the possible existence of an anomaly of cutaneous adrenergic nerves.

Aspirin↗
PubMed1979

[Functional peripheral ischemia].

The terminal circulation which plays an important role in the thermoregulation particularly at the periphery of the extremities is not infrequently irritated by false multifactorial regulations. As their sequela an increased tonus of vasoconstrictors and pathological vasodilatation lead to idiopathic and symptomatic functional angiolopathies. Transitions into organic vascular processes are no rarity. From the extreme functional behaviour patterns complaints and symptoms of the individual angiolopathies may be derived. Clinic, differential diagnostics and therapeutic possibilities of the angiopathic reaction position, of the acrocyanosis and its variants, of the intermittent functional acrosyndromes and of the erythromelalgia are treated from practical points of view. In contrast to the secondary functional angiolopathies, in which the basic disease is of decisive importance in questions concerning expertise, the idiopathic forms alone are scarcely of high significance concerning the restriction of the physical function.

Arterial Occlusive Diseases↗
PubMed1994

Erythromelalgic, thrombotic and haemorrhagic manifestations of thrombocythaemia.

OBJECTIVES: In order to document a relationship between platelet counts and clinical complication of thrombosis or bleeding in patients with myeloproliferative thrombocythaemia, 200 consecutive published cases of thrombocythaemia were reviewed for thrombotic or haemorrhagic manifestations and haematological findings at the time of the thrombohaemorrhagic complication. METHODS: Cases selected from the literature included all those complying with the diagnosis of thrombocythaemia as mandated by the Polycythemia Vera Study Group. One hundred consecutive cases of thrombocythaemia complicated by bleedings and one hundred consecutive cases of thrombocythaemia complicated by thrombosis were reviewed for clinical manifestations and haematological findings. RESULTS: In the reviewed cases of thrombocythaemia haemorrhagic complications (platelet count 2016 +/- 1070 x 10(9)/1) occurred at significantly (p < 0.001) higher degrees of thrombocytosis than thrombotic complications (platelet count 1110 +/- 477 x 10(9)/1). CONCLUSIONS: In the reviewed cases of thrombocythaemia arterial complications, especially erythromelalgia, can already be documented at platelet counts in excess of 400 x 10(9)/1, whereas bleeding complications usually occur at significantly higher degrees of thrombocytosis (> 1000 x 10(9)/1).

Adult↗
PubMed1993

Secondary erythermalgia in systemic lupus erythematosus; [comment].

Secondary erythermalgia, as a symptom of an underlying illness, is characterized by burning pain in the extremities together with local erythema and warmth. The onset and clinical symptomatology of secondary erythermalgia in a woman with a 20 year history of systemic lupus erythematosus (SLE) is described. Histopathologic examination of affected skin areas revealed an inflammatory process compatible with vasculitis. These findings completely differ from the histopathology in erythromelalgia and in primary erythermalgia. The erythermalgic symptoms completely resolved by treatment with prednisone. We conclude that secondary erythermalgia in SLE in our patient was associated with cutaneous vasculitis.

Erythromelalgia↗
PubMed2000

Hypnosis in dermatology.

BACKGROUND: Hypnosis is an alternative or complementary therapy that has been used since ancient times to treat medical and dermatologic problems. OBJECTIVE: To describe the various uses for hypnosis as an alternative or complementary therapy in dermatologic practice. METHODS: A MEDLINE search was conducted from January 1966 through December 1998 on key words related to hypnosis and skin disorders. RESULTS: A wide spectrum of dermatologic disorders may be improved or cured using hypnosis as an alternative or complementary therapy, including acne excoriée, alopecia areata, atopic dermatitis, congenital ichthyosiform erythroderma, dyshidrotic dermatitis, erythromelalgia, furuncles, glossodynia, herpes simplex, hyperhidrosis, ichthyosis vulgaris, lichen planus, neurodermatitis, nummular dermatitis, postherpetic neuralgia, pruritus, psoriasis, rosacea, trichotillomania, urticaria, verruca vulgaris, and vitiligo. CONCLUSION: Appropriately trained clinicians may successfully use hypnosis in selected patients as alternative or complementary therapy for many dermatologic disorders.

Complementary Therapies↗
PubMed1990

Clinical manifestations of essential thrombocythemia in young adults.

Essential thrombocythemia (ET) is a myeloproliferative disorder characterized by isolated overproduction of platelets, thrombohemorrhagic complications, and a median age of 50-60. When it occurs in younger patients, the incidence of complications has been reported to be quite low, with a good long-term prognosis. We report a retrospective review of 13 patients with ET between the ages of 22 and 35 in which 11 were symptomatic at diagnosis, with only one remaining asymptomatic during follow-up. Three patients presented with potentially life-threatening complications (two myocardial infarctions, one stroke), although no deaths were observed. The majority of the nonlife-threatening complications were vaso-occlusive in nature, including erythromelalgia and transient neurologic symptoms. We conclude that ET in young adults is not always a benign disease and that potentially life-threatening complications are not rare. The optimum approach to treatment in this or any other age group remains uncertain.

Adult↗