Dercum's disease or syndrome

Alternative eponyms

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Rare condition characterized by scattered areas of painful cutaneous nodules or fat accumulations.

Description

Rare condition characterized by scattered areas of painful cutaneous nodules or fat accumulations. Development of the nodular, tender lipomas is gradual with localization largely on the trunk and limbs with sparing of the face and hands.

Symptoms are asthenia, headache, frequently amenorrhoea and ecchymoses; diminution of sweating. Mental depression and deterioration have been observed in an appreciable number of these patients. Long progressive course. Death often caused by cardiac failure. Most cases of this rare disorder are sporadic but autosomal dominant inheritance has been documented.

It was earlier believed that it occurs chiefly in menopausal obese women of middle age, and sometimes in men. However, recent surveys of sufferers indicates that the disease causes the obesity, not the other way round, and that it occurs in men and women of all ages.

Dercum in 1888 first described the syndrome in a fifty-one year old woman and presented three more cases in 1892, writing:

    “Evidently the disease is not simple obesity. If so, how are we to dispose of the nervous elements present? Equally plain is it that we have not myxoedema to deal with. All of these cases lack the peculiar physiognomy, the spade-like hands, the infiltrated skin, the peculiar slowing of speech, and the host of other symptoms found in myxoedema. It would seem then, that we have here to deal with a connective tissue dystrophy, a fatty metamorphosis of various stages of completeness, occurring in separate regions, or at best unevenly distributed and associated with symptoms suggestive of an irregular and fugitive irritation of nerve-trunks – possibly a neuritis . . . Inasmuch as fatty swelling and pain are the most prominent features of the disease, I propose for it the name Adiposis Dolorosa.”

Bibliography

  • F. X. Dercum:
    A Subcutaneous connective tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema.
    University medical Magazine, Philadelphia, 1888-1889, 1: 140-150.
  • F. X. Dercum:
    Three cases of hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special nervous symptoms, adiposis dolorosa.
    American Journal of the Medical Sciences, Thorofare, N.J., 1892, 104: 521-535.
  • Roux and L. J. Vitaut:
    Maladie de Dercum (adiposis dolorosa).
    Revue neurologique, Paris, volume 9, 1901. Originally written as a thesis.

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