Emo syndromeE05.83

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

EMO syndromes; Exophthalmic Mycoedema Osteoarthropathy Syndrome; Exophthalmic myxoedema-circumscriptum-praetibial osteoarthropathia-hypertrophicans syndrome

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HistoryThis section has been translated automatically.

Thomas, 1933

DefinitionThis section has been translated automatically.

EMO = acronym for "exophthalmus, myxoedema, osteoarthropathy". Clinical symptom complex of endocrine orbitopathy with exophthalmos, prebital (also possible on hands and other localizations) myxedema and hypertrophic osteoarthropathy.

Occurrence/EpidemiologyThis section has been translated automatically.

For hyperthyroidism. Prevalence: about 1% of all hyperthyroid patients; no gender predisposition, no preference for a particular patient. Life stages

EtiopathogenesisThis section has been translated automatically.

Autoimmune disease; possibly increased stimulability of fibroblasts by autoantibodies. Pathogenetically, the lesions are caused by storage of acidic mucopolysaccharides in interfibrillary tissue. Pathogenetically, the pathological immunoglobulin LATS (long acting thyreoid stimulator) and EPS (exophthalmius producing sunstance) play an important role.

ManifestationThis section has been translated automatically.

Mainly in women with hyperthyroidism and Graves' disease

Clinical featuresThis section has been translated automatically.

The main characteristics of the EMO syndrome are a progressive exophthalmos (see also Endocrine Orbitopathy), relatively painless, skin-colored, doughy swellings of hands and feet with drumstick fingers and toes and watch glass nails. In addition, there are usually circumscribed, pretibial, doughy skin swellings with retracted hair follicles (peau d'orange) and usually mild hypertrichosis (see below: Myxoedema circumscriptum symmetricum praetibiale) as well as periosteal (radiologically detectable) bone formation. The symptoms of the disease usually appear a few weeks to years (!) after the treatment of hyperthyroidism, regardless of the previous form of treatment. As in endocrine orbitopathy, there is no correlation between the severity of EMO and current thyroid function.

LaboratoryThis section has been translated automatically.

Correlation with LATS, TSI, antibodies against thyroid microsomes and thyroglobulin (Note: Thyroid microsome antibodies (MAK or TMAK) are primarily directed against thyroid peroxidase (TPO), which catalyzes the iodination of tyrosine in thyroglobulin. Highly positive MAK together with TAK are characteristic for Hashimoto's thyroiditis).

Differential diagnosisThis section has been translated automatically.

TherapyThis section has been translated automatically.

Symptomatic therapy of the underlying disease.

Case report(s)This section has been translated automatically.

  • Case1: A 60-year-old female patient with a long known and treated Graves' disease with distinct endocrine orbitopathy (pronounced exophthalmos) has observed an increase in lower leg volumes with a distinct induration of the skin for several years. In addition, painless swelling of the hands and feet (steady increase in shoe size) as well as sensitivity disorders of the hands and feet were observed.
  • Findings: Conspicuous acropacity with flail fingers and watch glass nails. Pronounced coarse, painless pretiabial substance proliferation with clear emphasis on the follicular structures.
  • First clinical visual diagnosis: acromegaly.
  • Histology: A biopsy of the left big toe showed distinct dermal mucin deposits.
  • Laboratory: Highly positive thyroid microsomal antibodies (MAK or TMAK); growth hormone STH in the normal sense. No elevated somatomedin (IGF-I) levels. No indication of diabetes mellitus.
  • X-ray and CT of the skull: No enlargement of the sella!
  • X-ray of the extremities: evidence of hypertrophic osteoarthropathy.
  • Diagnosis: EMO syndrome
  • Therapy: Intensified treatment of the underlying disease.

LiteratureThis section has been translated automatically.

  1. Appelhans C et al (2004) Fibromatosis of the hand associated with EMO syndrome: a case report.
    BMC Dermatol 4:17.
  2. Brown-Falco O, Petzoldt D (1967) E.M.O. syndrome. Münch med Wschr 109: 1523-1529
  3. Imgrund M et al (2001) EMO syndrome as a late explanation for pretibial swelling. Rheumatology (Oxford) 40: 357-358
  4. Kato N et al (1991) A case report of EMO syndrome showing localized hyperhidrosis in pretibial myxedema. J Dermatol 18: 598-604
  5. Milkova L (2011) EMO syndrome under the picture of a pronounced acromegaly. Abstract CD 46th DDG meeting: P06/01
  6. Thomas HM Jr (1933) Acropachy, secondary subperiosteal new bone formation. Arch Internal Med 51: 571-588

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer