Adiposis dolorosa E88.2

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

Adiposis dolorosa; Anders Syndrome; Dercum disease; Dercum's disease; Dercum Vitaut Syndrome; Lipalgia; Neurolipomatosis; Obesalgia; Obesity dolorosa; Obesity tuberosa simplex; Other Disease

History
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Dercum, 1888

Definition
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Rare disease characterized by spontaneous or pressure painful lipomas, which is mainly observed in obese women in the menopause.

Other symptoms are fatigue, depression and other psychiatric and psychological abnormalities.

Classification
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Classification of Dercum's disease (n. Hansson E et al. 2012)

  • Subtype I Generalized diffuse form
  • Subtype II Generalised nodular form
  • Subtype III Localized nodular form
  • Subtype IV Juxtaarticular form

Etiopathogenesis
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Unknown. Mostly sporadically occurring, in some families autosomal dominant inherited.

Manifestation
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Mostly occurring in obese women in the menopause.

Localization
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Mainly located on the hull and acra.

Clinical features
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Localized obesity. Spontaneously developing, painful fat bulges covered by blue-red skin. Possibly pruritus, adynamic, apathy, mental disorders, especially emotional instability, depression, epilepsy, confusion and even dementia. Joint pain, nosebleeds.

Histology
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Normal fat tissue. No inflammatory changes.

Differential diagnosis
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Therapy
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A causal therapy is not known. In localized forms excision or liposuction.

Literature
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  1. Brodovsky S et al (1994) Adiposis dolorosa (Dercum's disease): 10-year follow-up. Ann Plast Surgery 33: 664-668
  2. Campen R (2001) Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 44: 132-136
  3. Dercum FX (1888) A subcutaneous connective tissue dystrophy of the arm and neck, associated with symptoms resembling myxedema. Univ Med Gaz Philadelphia 1: 140-150
  4. Dercum FX (1892) Three cases of a hitherto unclassified affection resembling in its great aspects obesity, but associated with special symptoms: adiposis dolorosa. On J Med Sci 104: 521-535
  5. Hansson E et al (2012) Review of Dercum's disease and proposal of diagnostic criteria, diagnosticmethods
    , classification and management. Orphanet J Rare Dis 7: 23.
  6. Kyllerman M (2002) Dysarthria, progressive parkinsonian features and symmetric necrosis of putamen in a family with painful lipomas (Dercum disease variant). Neuropediatrics 33: 69-72
  7. Steiner J (2002) Lipomatosis dolorosa--a frequently overlooked disease picture. Neurologist 73: 183-187
  8. Wollina U et al (2018) Differential diagnosis of lipedema and lymphedema. Dermatologist 69:1039-1047

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Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

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Last updated on: 29.10.2020