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[Gleich's syndrome].

S Emonet1, G Kaya, C Hauser

  • 1Unité d'Allergologie, Division d'Immunologie et d'Allergologie, Département de Médecine Interne, Hôpital Cantonal de Genève, 24, rue Micheli-du-Crest, CH-1211 Genève 14.

Annales De Dermatologie Et De Venereologie
|August 10, 2000
PubMed
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Gleich's syndrome, a rare condition causing episodic angioedema and hypereosinophilia, is benign but can be incapacitating. Glucocorticoids may help manage flare-ups of this unknown-etiology disease.

Area of Science:

  • Immunology
  • Allergy
  • Dermatology

Background:

  • Episodic angioedema with eosinophilia, also known as Gleich's syndrome, is a recently identified condition.
  • It is characterized by hypereosinophilia, episodic angioedema, and elevated immunoglobulin M levels.
  • The syndrome typically has a good prognosis with no organ involvement.

Observation:

  • A 39-year-old woman experienced 10 years of recurrent swelling and weight gain.
  • She presented with marked hypereosinophilia (up to 30,731 cells/mm³), elevated lactate dehydrogenase, eosinophil cationic protein, and immunoglobulin E.
  • Extensive investigations, including cardiac, pulmonary, and immunologic assessments, were negative, and bone marrow biopsy showed no abnormalities.

Findings:

  • The patient exhibited classic symptoms of Gleich's syndrome, including episodic angioedema and significant hypereosinophilia.

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  • Laboratory findings revealed elevated inflammatory markers and immunoglobulins.
  • Despite the severity of symptoms during episodes, all organ functions remained normal.

    • Implications:

    • Gleich's syndrome, while benign, can be debilitating for patients.
    • The etiology of this syndrome remains unknown.
    • Systemic glucocorticoids show potential in managing and controlling symptomatic flare-ups.