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Urine stain that darkens after washing
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  • Pink staining of diapers can be caused by: (1) uric acid crystals, (2) hemoglobinuria, (3) dyes from sweets, (4) anthocyanins from beetroot or berries, (5) drugs, (6) porphyria, (7) contamination with the Serratia marcesans bacterium, and (8) alkaptonuria. [101]
  • Fluorescence of a diaper under Wood's light is typically encountered only in congenital erythropoietic porphyria, one of the rarest porphyrias. [103]
  • This old book contains a paragraph about the "blue diaper syndrome," linking it to idiopathic hypercalcemia in infants. The metabolic defect involves tryptophan metabolism and results in a high urinary excretion of indican and indole derivatives, one of which turns blue when oxidized. [104]
  • Describes the case of Harwa, an Egyptian granary custodian who died about 1500 BC, but spent 60 years of this century on display in Chicago's Field Museum of Natural History. [107]
  • More about Harwa. [108]
  • A wonderful article, full of clinical detail. One can almost imagine Osler bounding out of his chair to stop the patient and his pigmented ears from leaving the examination room!
    • "As he left the room my attention was directed to the deep blue colour of the inner surface of the ears.... From behind and along the free border of the helix the skin looks normal but when looked at from inside there is a remarkable blue-black discolouration, exactly like that produced by dilated veins, as Albrecht remarked. It is deepest in the concha and extends along the antihelix but not to the helix. I did not recognise at first that the pigmentation was in the cartilage. In certain positions and when the light falls into the ears the colour at once attracts attention."
    • "At my first examination I was impressed by a remarkable appearance of the sclerotics which showed small V shaped areas of deep pigmentation near the cornea.... I searched the literature [for] an explanation but without avail.... The exposed V-shaped portions are of a deep black colour, not in the entire extent, as there are areas of normal colour. The staining is in the sclerotic coat, not in the conjunctiva, and it does not extend to the covered parts of the eyeballs.... There is nothing special to be noticed about the other parts of the eyes. The tarsal cartilages are not affected."
  • "Exogenous ochronosis due to absorption of phenol used to dress wounds and ulcers, formerly frequent, has probably disappeared since the introduction of modern antiseptics." [p. 129] [110]
    Footnotes in Print Edition[Top] [Nav]
    (1) The Metabolic Basis of Inherited Disease. 4th ed.
  Stanbury JB, et al. New York: McGraw-Hill, 1978: 268-282.
    (2) Alkaptonuria.
  Srsen S.   Johns Hopkins Med J 1979 Dec;145(6):217-26.  Pubmed+Abstract  Similars
    (3) McKusick 203500 = alkaptonuria.
    (4) Alkaptonuria and aortic stenosis [letter].
  Vlay SC, Hartman AR, et al.   Ann Intern Med 1986 Mar;104(3):446.  Pubmed  Similars
    New References[Top] [Nav]

    101.Pink napkins--presenting feature in a case of alkaptonuria.
  Sutherland DA, Nicol AD, et al.   J Inherit Metab Dis 1984;7(2):56.  Pubmed  Similars
    102.Images in clinical medicine. Diaper diagnosis of porphyria [see comments].
  Pollack SS, Rosenthal MS.   N Engl J Med 1994 Jan 13;330(2):114.  Pubmed  Similars
    103.A porphyrin-soaked diaper [letter; comment].
  Pierach CA, Ippen H.   N Engl J Med 1994 Jun 9;330(23):1690.  Pubmed  Similars
    104.Rare Diseases and Lesions.
  Bean WB. Springfield, IL: Thomas, 1967: 167.

Other clinical manifestations
    105.Cardiovascular findings in alkaptonuric ochronosis.
  Kragel AH, Lapa JA, et al.   Am Heart J 1990 Dec;120(6 Pt 1):1460-3.  Pubmed  Similars
    106.Ochronosislike pigmentation from hydroquinone bleaching creams in American blacks.
  Hoshaw RA, Zimmerman KG, et al.   Arch Dermatol 1985 Jan;121(1):105-8.  Pubmed+Abstract  Similars

    107.Biochemical identification of homogentisic acid pigment in an ochronotic egyptian mummy.
  Stenn FF, Milgram JW, et al.   Science 1977 Aug 5;197(4303):566-8.  Pubmed+Abstract  Similars
    108.Characterization of mummy bone ochronotic pigment.
  Lee SL, Stenn FF.   JAMA 1978 Jul 14;240(2):136-8.  Pubmed+Abstract  Similars
    109.Ochronosis: the pigmentation of cartilages, sclerotics, and skin in alkaptonuria.
  Osler W.   Lancet. 1904;I:10-11.
    110.Pigmentation of the skin.
  Jeghers H.   N Engl J Med. 1944;231:88-100,122-136,181-189.
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    ©1986-2000 John Sotos, MD. All rights reserved.  Last updated 16:34 PDT on July 4, 2000.[Top]

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