Medical Mystery — The Answer
http://www.100md.com
《新英格兰医药杂志》
To the Editor: The medical mystery in the October 7 issue1 involved a 16-year-old white boy who presented with generalized argyria after ingesting a silver-containing dietary supplement for approximately two years. The supplement was packaged so that it was identical to bottled water and was touted as a preventive for everyday infections. Pigmentary changes began on the boy's cheeks and progressed to involve his entire body within one year. Because of his graying complexion, a workup was performed for Addison's disease and cyanosis. All the results were negative or normal. There was no evidence of neuropathy or seizures.
A physical examination revealed striking, slate-blue–gray hyperpigmentation of the boy's entire body, which was accentuated on the sun-exposed areas (Figure 1), including the head, neck, and oral mucosa. The backs of the hands, nail beds, and conjunctivae were also involved. Microscopical examination of a skin-biopsy specimen revealed finely granular, dark-brown pigment in the basement-membrane zone surrounding sweat glands and in connective-tissue sheaths around hair follicles (Figure 2). The serum silver level was markedly elevated, at 209 ng per millimeter (normal range, 0 to 14). These findings are consistent with the diagnosis of generalized argyria.
Figure 1. A 16-Year-Old Boy with Hyperpigmentation of the Skin.
Figure 2. Skin-Biopsy Specimen (Hematoxylin and Eosin).
Dark-brown pigment (arrows) is present in the basement-membrane zone surrounding sweat glands and in connective-tissue sheaths around hair follicles.
Scott C. Wickless, D.O.
Oakwood Health System
Dearborn, MI 48124
wickless@umich.edu
Tor A. Shwayder, M.D.
Henry Ford Health System
Detroit, MI 48202
References
Wickless SC, Shwayder TA. A medical mystery. N Engl J Med 2004;351:1547-1547.
Editor's note: Even though this was a difficult medical mystery, 1077 responses were received from readers in 68 countries. Thirteen percent of the respondents diagnosed an ingestion; half of these respondents recognized the slate-blue discoloration as due to the accumulation of silver in the skin and correctly identified the condition as argyria. The key to making the correct diagnosis was the ability to see the slate-blue hue of the dermal pigmentation, which was carefully controlled in the print edition but was subject to a variety of uncontrollable factors in the electronic edition. The incidence of argyria has decreased as occupational, medicinal, and cosmetic exposure to silver has diminished.
Other ingested substances that were suggested included carotene, minocycline, chrysiasis, bismuth, and amiodarone. Common alternative diagnoses proposed were Addison's disease (45 percent of respondents), hemochromatosis (24 percent of respondents), Wilson's disease, porphyria, and methemoglobinemia; some respondents even suggested tanning of the skin. A few respondents suggested that the mother had vitiligo or albinism. One very perceptive respondent commented, "Mom's arm around her son's shoulders conveys her affection. Her Mona Lisa smile also conveys her love as well as her chagrin at her misguided efforts to boost his immune system by giving him silver supplements."
Lindsey R. Baden, M.D.
A physical examination revealed striking, slate-blue–gray hyperpigmentation of the boy's entire body, which was accentuated on the sun-exposed areas (Figure 1), including the head, neck, and oral mucosa. The backs of the hands, nail beds, and conjunctivae were also involved. Microscopical examination of a skin-biopsy specimen revealed finely granular, dark-brown pigment in the basement-membrane zone surrounding sweat glands and in connective-tissue sheaths around hair follicles (Figure 2). The serum silver level was markedly elevated, at 209 ng per millimeter (normal range, 0 to 14). These findings are consistent with the diagnosis of generalized argyria.
Figure 1. A 16-Year-Old Boy with Hyperpigmentation of the Skin.
Figure 2. Skin-Biopsy Specimen (Hematoxylin and Eosin).
Dark-brown pigment (arrows) is present in the basement-membrane zone surrounding sweat glands and in connective-tissue sheaths around hair follicles.
Scott C. Wickless, D.O.
Oakwood Health System
Dearborn, MI 48124
wickless@umich.edu
Tor A. Shwayder, M.D.
Henry Ford Health System
Detroit, MI 48202
References
Wickless SC, Shwayder TA. A medical mystery. N Engl J Med 2004;351:1547-1547.
Editor's note: Even though this was a difficult medical mystery, 1077 responses were received from readers in 68 countries. Thirteen percent of the respondents diagnosed an ingestion; half of these respondents recognized the slate-blue discoloration as due to the accumulation of silver in the skin and correctly identified the condition as argyria. The key to making the correct diagnosis was the ability to see the slate-blue hue of the dermal pigmentation, which was carefully controlled in the print edition but was subject to a variety of uncontrollable factors in the electronic edition. The incidence of argyria has decreased as occupational, medicinal, and cosmetic exposure to silver has diminished.
Other ingested substances that were suggested included carotene, minocycline, chrysiasis, bismuth, and amiodarone. Common alternative diagnoses proposed were Addison's disease (45 percent of respondents), hemochromatosis (24 percent of respondents), Wilson's disease, porphyria, and methemoglobinemia; some respondents even suggested tanning of the skin. A few respondents suggested that the mother had vitiligo or albinism. One very perceptive respondent commented, "Mom's arm around her son's shoulders conveys her affection. Her Mona Lisa smile also conveys her love as well as her chagrin at her misguided efforts to boost his immune system by giving him silver supplements."
Lindsey R. Baden, M.D.