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- Rare
- characterized by
- scattered areas of painful cutaneous nodules or fat accumulations
- development of the nodular, tender lipomas is gradual
- localization largely to forearms and thighs
- occurs chiefly in menopausal obese women of middle age and
- may be familial
Symptoms
- asthenia,
- headache,
- frequently amenorrhoea and ecchymoses;
- diminution of sweating.
- Mental depression and deterioration have been observed in an appreciable
number of these patients.
Family History
- Most cases of this rare disorder are sporadic but autosomal dominant
inheritance has been documented.
Prognosis
- Long progressive course.
- Death often caused by cardiac failure.
Early Description
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.”
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