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Stiff joints and thickened skin after an episode of unusual physical exertion
    Differential Diagnosis[Top] [Nav]
eosinophilic fasciitis (Shulman disease)
    Discussion -- not available online[Top] [Nav]
    Notes[Top] [Nav]
       *Unusual thickening of the skin is no longer a zebra, or at least it wasn't in the late 1980s and early 1990s. In 1989 a new syndrome was discovered: eosinophilia-myalgia linked with ingestion of L-tryptophan. One of the characteristic findings in this syndrome is progressive thickening of the skin, sometimes occurring after physical exertion. The Varga paper and the Troy editorial nicely discuss the eosinophilia-myalgia syndrome's relationship to eosiniphilic fasciitis, the toxic oil syndrome, scleroderma (systemic and morphea), and even carcinoid. [Some patients with the carcinoid syndrome develop skin thickening, thought due to high levels of serotonin. Tyrptophan is metabolized to serotonin.]
    Update Comments[Top] [Nav]
  • Half of the Mayo Clinic's patients with eosinophilic fasciitis had been exposed to tryptophan. [101]
  • The pathologist diagnosed eosinophilic fasciitis, but this was later revised (see letter below). [104]
  • The patient in CPC 4-1990 had long used tryptophan as a sleeping aid, until mid-1987. Her first visit to the arthritis clinic was in August 1988. She was found dead at home in January 1990. [105]
  • Lyme disease can express a syndrome of diffuse fasciitis and peripheral eosinophilia. [106]
  • Old letter reporting prompt improvement in eosinophilic fasciitis in response to cimetidine treatment. [107]
  • Studied the patients in one psychiatrist's practice in South Carolina. Opines that the CDC diagnostic criteria for eosinophilia-myalgia syndrome do not identify all affected patients. [110]
  • First report of the syndrome. [113]
  • Reminds that other toxic agents and diseases may be associated with scleroderma-like skin thickening: polyvinylchloride, chlorinated hydrocarbons, silicone implants, porphyria cutanea tarda, phenylketonuria, and some of the glycogenoses. [114]
  • Implicates the tryptophan manufactured by one company in Japan. [116]
  • Scleroderma-like skin thickening is part of this syndrome. [117]
    Footnotes in Print Edition[Top] [Nav]
    (1) Clinical conferences at The Johns Hopkins Hospital.
  Johns Hopkins Med J 1981 Feb;148(2):81-6.  Pubmed  Similars
    (2) Diffuse fasciitis with eosinophilia in childhood.
  Sills EM.   Johns Hopkins Med J 1982 Nov;151(5):203-7.  Pubmed+Abstract  Similars
    (3) Textbook of Rheumatology. 2nd ed.
  Kelley WN, et al. Philadelphia: Saunders, 1985: 1195-1196.
    New References[Top] [Nav]

Eosinophilic fasciitis
    101.The eosinophilia-myalgia syndrome: the Los Alamos Conference.
  Hertzman PA, Falk H, et al.   J Rheumatol 1991 Jun;18(6):867-73.  Pubmed+Abstract  Similars
    102.Eosinophilic fasciitis associated with use of L-tryptophan: a case- control study and comparison of clinical and histopathologic features.
  Martin RW, Duffy J, et al.   Mayo Clin Proc 1991 Sep;66(9):892-8.  Pubmed+Abstract  Similars
    103.L-tryptophan ingestion associated with eosinophilic fasciitis but not progressive systemic sclerosis.
  Freundlich B, Werth VP, et al.   Ann Intern Med 1990 May 15;112(10):758-62.  Pubmed+Abstract  Similars
    104.Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 4-1990. A 53-year-old woman with cutaneous thickening and eosinophilia.
  N Engl J Med 1990 Jan 25;322(4):252-61.  Pubmed  Similars
    105.Case 4-1990: eosinophilic fasciitis [letter].
  Gorn A.   N Engl J Med 1990 Mar 29;322(13):931.  Pubmed  Similars
    106.Identification of Borrelia burgdorferi in diffuse fasciitis with peripheral eosinophilia: borrelial fasciitis.
  Granter SR, Barnhill RL, et al.   JAMA 1994 Oct 26;272(16):1283-5.  Pubmed+Abstract  Similars
    107.Cimetidine and eosinophilic fasciitis [letter].
  Garcia-Morteo O, Maldonado-Cocco JA, et al.   Ann Intern Med 1984 Feb;100(2):318-9.  Pubmed  Similars

    108.The natural history of eosinophilia-myalgia syndrome in a tryptophan- exposed cohort in South Carolina.
  Sullivan EA, Kamb ML, et al.   Arch Intern Med 1996 May 13;156(9):973-9.  Pubmed+Abstract  Similars
    109.Repeated coronary artery spasm in a young woman with the eosinophilia- myalgia syndrome.
  Hertzman PA, Maddoux GL, et al.   JAMA 1992 Jun 3;267(21):2932-4.  Pubmed+Abstract  Similars
    110.Eosinophilia-myalgia syndrome in L-tryptophan-exposed patients [see comments].
  Kamb ML, Murphy JJ, et al.   JAMA 1992 Jan 1;267(1):77-82.  Pubmed+Abstract  Similars
    111.The cause and pathogenesis of the eosinophilia-myalgia syndrome [see comments].
  Varga J, Uitto J, et al.   Ann Intern Med 1992 Jan 15;116(2):140-7.  Pubmed+Abstract  Similars
    112.Natural history of the eosinophilia-myalgia syndrome.
  Culpepper RC, Williams RG, et al.   Ann Intern Med 1991 Sep 15;115(6):437-42.  Pubmed+Abstract  Similars
    113.Eosinophilia-myalgia syndrome--New Mexico.
  MMWR Morb Mortal Wkly Rep 1989 Nov 17;38(45):765-7.  Pubmed  Similars
    114.Eosinophilia-myalgia syndrome [editorial; comment].
  Troy JL.   Mayo Clin Proc 1991 May;66(5):535-8.  Pubmed  Similars
    115.Eosinophilia-myalgia syndrome. A clinical case series of 21 patients. New Mexico Eosinophilia-Myalgia Syndrome Study Group.
  Philen RM, Eidson M, et al.   Arch Intern Med 1991 Mar;151(3):533-7.  Pubmed+Abstract  Similars
    116.An investigation of the cause of the eosinophilia-myalgia syndrome associated with tryptophan use [see comments].
  Belongia EA, Hedberg CW, et al.   N Engl J Med 1990 Aug 9;323(6):357-65.  Pubmed+Abstract  Similars

Toxic oil syndrome
    117.Clinical epidemiology of toxic-oil syndrome. Manifestations of a New Illness.
  Kilbourne EM, Rigau-Perez JG, et al.   N Engl J Med 1983 Dec 8;309(23):1408-14.  Pubmed+Abstract  Similars
    118.3-(Phenylamino)alanine, a novel aniline-derived amino acid associated with the eosinophilia-myalgia syndrome: a link to the toxic oil syndrome? [see comments].
  Mayeno AN, Belongia EA, et al.   Mayo Clin Proc 1992 Dec;67(12):1134-9.  Pubmed+Abstract  Similars
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    ©1986-2000 John Sotos, MD. All rights reserved.  Last updated 16:33 PDT on July 4, 2000.[Top]

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