Acrodynia (or pink disease, erythredema, erythredema polyneuropathy, Bilderbeck's, Selter's, Swift's and Swift-Feer disease) refers to a condition of pain and dusky pink discoloration in the hands and feet most often seen in children chronically exposed to heavy metals, especially mercury.

The word is derived from the Greek, where ακρος means high (as in:in an extremity) and οδυνη means pain. As such, it might be (erroneously) used to indicate that a patient has pain in the hands or feet. However, acrodynia is a disease rather than a symptomHorowitz Y, Greenberg D, Ling G, Lifshitz M. Acrodynia: a case report of two siblings. Arch Dis Child 2002; 86: 453. PMID 12023189.

Causes

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Mercury compounds like calomel were historically used for various medical purposes: as laxatives, diuretics, antiseptics or antimicrobial drugs for syphilis, typhus and yellow feverBeck C, Krafchik B, Traubici J, Jacobson S. Mercury intoxication: it still exists. Pediatr Dermatol 2004; 21: 254-9. PMID 15165207 . Teething powders were are widespread source of mercury poisoning until the recognition of mercury toxicity in the 1940s.

However, mercury poisoning and acrodynia still exist today Weinstein M, Bernstein S. Pink ladies: mercury poisoning in twin girls. CMAJ 2003; 168: 201. PMID 12538551. Modern sources of mercury intoxication include broken thermometers Torres AD, Rai AN, Hardiek ML. Mercury intoxication and arterial hypertension: report of two patients and review of the literature. Pediatrics 2000; 105: E34. PMID 10699136.

Symptoms

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Besides peripheral neuropathy (presenting as paresthesia or itching, burning or pain) and discoloration, swelling (edema) and desquamation may occur.

Since mercury blocks the degradation pathway of catecholamines, epinephrine excess causes profuse sweating (diaphora), tachycardia, salivation and elevated blood pressure. Mercury is suggested to inactivate S-adenosyl-methionine, which is necessary for catecholamine catabolism by catechol-o-methyl transferase.

Affected children may show red cheeks and nose, red (erythematous) lips, loss of hair, teeth, and nails, transient rashes, hypotonia and photophobia. Other symptoms may include kidney disfunction (e.g. Fanconi syndrome) or neuropsychiatric symptoms (emotional lability, memory impairment, insomnia).

Thus, the clinical presentation may resemble pheochromocytoma or Kawasaki disease.

There is some evidence that the same mercury poisoning may predispose to Young's syndrome (men with bronchiectasis and low sperm count)Hendry WF, A'Hern FPA, Cole PJ. Was Young's syndrome caused by mercury exposure in childhood? BMJ 1993;307:1579-82. PMID 8292944.

Therapy

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The standard of care is discontinuation of the environmental exposure, and chelation therapy (with EDTA or maybe better, DMSA).

References

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Occupational diseases | Toxicology | Pediatrics