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At least 19 recordsLinked to original sources

Pathogenesis of erythromelalgia.

A defective prostaglandin metabolism in patients with erythromelalgia may explain several of the clinical features of this condition, such as the red discoloration and burning sensations of the skin. In two patients with erythromelalgia a grossly abnormal bullous reaction to intradermally injected PGE1, PGE2, and PGF1alpha occurred, whereas a normal reaction appeared after injection of histamine, serotonin, and bradykinin. Furthermore, prostaglandin-like material was detected in increased concentration in skin perfusates from these patient. In PGE1-equivalents the concentration amounted to 2.0 and 3.2 ng/ml of the original perfusate, as compared to 0.1 ng/ml in normal skin. The capacity of synthesizing prostaglandins was increased in skin biopsy material from both patients. At least part of the therapeutic effect of aspirin in these patients may be due to the influence of this drug on prostaglandin metabolism.

Adult

[Primary erythromelalgia].

Erythromelalgia is a rare disease characterized by intense erythema, burning pain and increased temperature in the distal of the extremities. Primary forms and secondary forms have been described, most commonly with essential thrombocythemia and policythemia vera. The authors describe a fifteen year old patient with primary erythromelalgia and discuss the pathogenic, clinical and therapeutic features of this disease.

Adolescent

Erythromelalgia.

Erythromelalgia is an extraordinary disease which remains elusive in its pathophysiology and management. Victims suffer intense burning and redness of the hands and feet. In what appears to be the antithesis of Raynaud's disease, the pain is relieved by emersion in cold. A child with erythromelalgia is described whose symptoms began at age 3 years. Pharmacological management trials and thermography are incorporated in the report.

Child, Preschool

Further characterization of the biological and pathogenic properties of erythromelalgia-related poxviruses.

Six isolates of erythromelalgia-related poxvirus (ERPV) were characterized with respect to host range, c.p.e. and inclusions, pock formation on chorioallantoic membrane (CAM), morphogenesis, serological reactivity, pathogenesis in animals and DNA restriction fragment profile. The results suggest that ERPV is either a new member of the Orthopoxvirus genus or a subspecies of ectromelia virus. Evidence is provided that (i) ERPV has a wide host range in vitro in which characteristic viral c.p.e. and inclusion bodies are induced; (ii) ERPV, unlike ectromelia virus, causes the formation of tiny greyish-white pocks on CAM both at 34 degrees C and 39 degrees C; (iii) eosinophilic A-type inclusions of ERPV do not contain viral particles; (iv) ERPV isolates are neutralized by both rabbit anti-vaccinia virus and mouse anti-ectromelia virus sera, but not vice versa; (v) young rabbits are not susceptible to ERPV by skin and/or corneal scratch infection even though ERPV is lethal for mice by intraperitoneal inoculation; (vi) the HindIII and SalI fragment profiles of ERPV P-4 DNA are similar to, but obviously different from, those of Chinese ectromelia virus. These biological and pathogenic characteristics of ERPV are distinguishable from those of other members of the genus Orthopoxvirus currently described in the literature.

Animals

Erythromelalgia.

Erythromelalgia is an extraordinary disorder of unknown etiology and pathophysiology that resembles the post-traumatic reflex dystrophy syndromes but has not been described previously in the orthopedic literature. Its distinctive triad of intense burning extremity pain associated with erythema and increased skin temperature are diagnostic. Primary or idiopathic and a secondary or associated form have been identified. The latter occurs in association with an underlying disease process, especially myeloproliferative disorders. Treatment with pharmacologic agents and surgery are ineffective except in the secondary group where treatment of the associated disorder generally results in a remission. Symptoms in the primary group can be minimized by appropriate environmental control with cooling and avoiding heat-producing situations that would raise skin temperature above a critical thermal threshold.

Adolescent

[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

Long-term treatment of parkinsonism with bromocriptine.

92 patients with parkinsonism have been treated with bromocriptine for up to 30 months. 48 continue to receive bromocriptine with benefit; of these, 35 take bromocriptine (mean dose 53 mg daily) in combination with levodopa and 13 take bromocriptine (mean dose 45 mg daily) without levodopa. In those who were originally on levodopa, addition of bromocriptine allowed a mean 41% reduction in the dose of levodopa; the largest group of patients to benefit from bromocriptine entered the study because of excessive dyskinesia or "on-off" phenomena induced by levodopa. In 40 patients bromocriptine was stopped because of adverse reactions, absence of therapeutic response, or non-compliance with the protocol. The main problems were psychiatric disturbance (8 patients) and erythromelalgia (7 patients); these effects tended to occur late (mean 6 months and 10 months, respectively) and with high dosage (mean 66 mg and 115 mg daily). Other frequent adverse effects were dizziness and nausea; these began considerably earlier (at 2 months and 1 month) and with much lower dosage (31 mg and 12 mg daily). 4 patients died, for reasons apparently unrelated to therapy.

Bromocriptine

[Paroxystic vasomotor skin manifestations (author's transl)].

Paroxystic vasomotor skin manifestations are provoked by various etiologies. Widespread or generalized vasomotor skin manifestations may be induced by a physiological reaction (emotinal flushing), by a drug (vasodilator drugs, antabuse, antidiabetic, sulfonamides), by a discharge of histamine (urticaria, mastocytosis) or by an hypersecretion of serotonin (dumping-syndrome, carcinoid syndrome). They may be caused by an endocrinopathy (menopause, hyperthyroidism, hypoglycaemia, medullary thyroid carcinoma, pheochromocytoma, endocrine pancreas, carcinoma). More rarely vasomotor troubles happen in homocystinuria, inhalation of a toxic (trichlorethylen, calcic cyanamid) and exceptionally in some immunohaematologic diseases. Main localized vasomotor skin manifestations observed are dermographism, facial flushing (Sluder's syndrome, cluster headaches, Frey's syndrome, Riley-Day's syndrome) and acral syndromes (Raynaud's phenomenon, erythromelalgia).

Amidohydrolases

Erythermalgia with vasculitis: a review.

Erythermalgia is a condition of the extremities characterized by redness, increased temperature, and burning pain. A case of erythermalgia and coincident vasculitis of the feet is reported. The literature on the subject is reviewed, and a possible mechanism of pathogenesis is discussed.

Erythromelalgia

Verapamil-induced secondary erythermalgia.

A 59-year-old man developed red, swollen and warm feet accompanied by intermittent burning pain during treatment for cardiac failure and arrhythmias with several drugs including verapamil. The condition gradually worsened until there was persistent disabling burning pain and severe erythema and swelling of the feet. Aspirin and other analgesics were ineffective in relieving the discomfort. Histopathology of punch biopsies showed a mild perivascular mononuclear infiltrate and moderate perivascular oedema. Within 2 weeks of stopping verapamil the burning pain, erythema, and swelling of the feet had resolved. The clinical features and subsequent course are consistent with a diagnosis of erythermalgia secondary to verapamil.

Erythromelalgia

Burning feet.

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Erythromelalgia