Eosinophilic myalgie syndromeM35.8
Synonym(s)
Eosinophilia-Myalgie-Syndrome L-Tryptophan-related; Eosinophilic Myalgia Syndrome; L-tryptophan-related eosinophilia-myalgie syndrome; Myalgia syndrome eosinophilic
DefinitionThis section has been translated automatically.
Disease characterized by generalized myalgia and eosinophilia (more than 1000 eosinophils/ml), usually occurring 1 to 12 months after taking L-tryptophan. An infection or tumour as a trigger of the disease must be excluded.
EtiopathogenesisThis section has been translated automatically.
- Contamination of the L-tryptophan during the genetic engineering production by the Japanese manufacturer Showa-Denko (90% of the world market): Sog. "Peak E" corresponds to a modified amino acid, the 1,1'-ethylidene bis(tryptophan). In addition, there seems to be an individual genetic predisposition to disorders in the tryptophan metabolism.
- Tryptophan induces interleukin-3 and -4 as well as GM-CFS with activation of eosinophilic granulocytes, which in turn enzymatically (MBP, ECP, EDN) activate fibroblasts, endothelial cells and nerve cells for synthesis or damage them toxicly, resulting in fibrosis, ischemia and neuropathy.
Clinical featuresThis section has been translated automatically.
Myalgia (100% of patients), arthralgia (73%), weakness, dyspnea (59%), volatile maculopapular or urticarial eruptions (60%), generalized itching, morphea-like foci (32%) and diffuse alopecia (28%). In the course of the disease, an early phase of edema with peripheral edema (59%) and periorbital edema (28%) is separated from a late phase of connective tissue proliferation with organ manifestations such as pulmonary hypertension, cardiac arrhythmia, myopathies, hyperthyroidism.
LaboratoryThis section has been translated automatically.
More than 1000/μl Eosinophils (100%), leucocytosis (85%), aldolase elevation (46%), increase in liver function values (43%), BSG acceleration (33%).
HistologyThis section has been translated automatically.
Thickening and inflammation of the deep dermis through accumulation of collagen and mucopolysaccharides. Infiltration with mononuclear cells and eosinophil granulocytes. Vimentin-positive and Mac-387-negative cells (fibroblasts) appear.
Differential diagnosisThis section has been translated automatically.
TherapyThis section has been translated automatically.
- Stopping the tryptophan; there is no spontaneous improvement under this alone, therefore additionally apply systemic glucocorticoids (e.g. Decortin H). Start with 0.5-1.0 mg/kg bw/day, then slowly reduce to 6-10 mg/day. After 3-6 months, weaning is usually successful.
- Physiotherapy and massages to prevent contractures.
Progression/forecastThis section has been translated automatically.
Slow regression, possible recurrence. Since the distribution of tryptophan-containing preparations was banned by the Federal Health Office in 1990, only sporadic occurrence of the disease can be expected.
LiteratureThis section has been translated automatically.
- Belongia EA et al (1990) An investigation of the cause of the eosinophilia-myalgia syndrome associated with tryptophan use. N Engl J Med 323: 357-366
- Centers for Disease Control (1989) Eosinophilia-myalgia syndrome: New Mexico. MMWR 38: 765-767
- Hertzman PA et al (2001) Rigorous new approach to constructing a gold standard for validating new diagnostic criteria, as exemplified by the eosinophilia-myalgia syndrome. Arch Internal Med 161: 2301-2306
- Kaufman LD et al (1990) Cutaneous manifestations of the L-tryptophan-associated eosinophilia-myalgia syndrome: A spectrum of sclerodermatous skin disease. J Am Acad Dermatol 23: 1063-1069
- Margolin L (2003) Non-L-tryptophan related eosinophilia-myalgia syndrome with hypoproteinemia and hypoalbuminemia. J Rheumatol 30: 628-629
- Mensing H et al (1992) The eosinophilia-mayalgie syndrome. Dermatologist 43: 436-440
- Silver RS et al (1990) Scleroderma, fasciitis and eosinophilia associated with the ingestion of tryptophan. N Engl J Med 322: 874-881
- Varga J et al (1993) L-Tryptophan and the Eosinophilia-myalgia syndrome; current understanding of the etiology and pathogenesis. J Invest Dermatol 100: 97-105