Erythromelalgia

Synonym(s)

Erythermalgia

Definition

Disorder characterized by paroxysmal burning pain, warmth, and redness of the extremities

Pathogenesis

Unclear; arteriolar fibrosis and occlusion with platelet thrombi often present; prostaglandins and cyclooxygenase involved

Clinical manifestation

Most cases primary (idiopathic); secondary form sometimes precede myeloprolifera-tive disorder with thrombocytosis; dramatic relief with aspirin typical of this type and useful in diagnosis; painful, warm extremities brought on by warming or dependency, lasting minutes to days, and relieved by cooling; lower extremities affected more often than upper extremities; symptoms worsening with warming of extremity or placing of extremity in a dependent position; symptoms sometimes decrease with cooling and elevation of extremity; no symptoms or signs between attacks

Differential diagnosis

Raynaud phenomenon; reflex sympathetic dystrophy; cellulitis; vasculitis; frostbite

Therapy

Cooling or elevating extremity to relieve symptoms of an attack*; aspirin 500 mg PO as needed; chemotherapy for myeloprolifer-ative disorder

References

Cohen JS (2000) Erythromelalgia: new theories and new therapies. Journal of the American Academy of Dermatology 43(5 Pt i):84i-847

226 Erythromycin, systemic

Erythromycin, systemic

Trade name(s)

Eryc; E-mycin; PCE; EES; Ilosone

Generic available

Drug class

Macrolide antibiotic

Mechanism of action

Inhibition of RNA-dependent protein synthesis by binding to the 50S subunit of the ribosome

Dosage form

250 mg, 333 mg, 400 mg, 500 mg tablet

Dermatologic indications and dosage

See table

Common side effects

Cutaneous: urticaria or other vascular reaction, stomatitis

Gastrointestinal: nausea and vomiting, diarrhea, abdominal cramps, jaundice Laboratory: elevated liver enzymes; eosi-nophilia

Serious side effects

Bone marrow: suppression Cardiovascular: arrhythmias, hypotension Cutaneous: anaphylaxis, Stevens-Johnson syndrome

Drug interactions

Amiodarone; amitriptyline; budesonide; buspirone; carbamazepine; clozapine; oral contraceptives; cyclosporine; digoxin; ergot alkaloids; methadone; phenytoin; pimoz-ide; protease inhibitors; quinidine; statins; tacrolimus; theophylline; valproic acid; vinca alkaloids; warfarin

Contraindications/precautions

Hypersensitivity to drug class or component; caution in patients with myasthenia gravis or impaired liver function

References

Alvarez-Elcoro S, Enzler MJ (1999) The mac-rolides: erythromycin, clarithromycin, and azithromycin. Mayo Clinic Proceedings 74(6):613-

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