Adiposis dolorosa E88.2

Dercum, 1888

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 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

m:w=5-30:1 (!) (Bardazzi F et al. 2024)

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

Mostly occurring in obese women during the menopause.

Mainly located on the hull and acra.

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.

Normal fat tissue. No inflammatory changes.

Painful angiolipomas (increased vascular markings)

Elephantiasis: mainly affects the lower extremities. Limb-shaped swellings; usually only tension pain

Lipoedema: Always symmetrical, disproportionate increase in fat on the arms and legs. Rather a feeling of tension; typical is a "caliber jump" to the adjacent healthy region ("muff", "Turkish pants phenomenon", "collar formation"). Hands and feet always remain free
Non-painful lipoma: soft, non-pressurized lump, no spontaneous pain

Lipoedema syndrome, painful: Affects mainly the lower extremity

Multiple symmetrical lipomatosis: Soft non-pressurized lipoma masses, no spontaneous pain

Hematomas of different age and coloration: Characteristic hematoma coloration. remembered trauma

Panniculitis of various origins: mostly painful, nodular changes in the fatty tissue.

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

1. Bardazzi F et al. (2024)* More than mere lipomas? J Dtsch Dermatol Ges 22:601-603.
2. Brodovsky S et al. (1994) Adiposis dolorosa (Dercum's disease): 10-year follow-up. Ann Plast Surg 33: 664-668
3. Campen R (2001) Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 44: 132-136
4. 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
5. Dercum FX (1892) Three cases of a hitherto unclassified affection resembling in its grosser aspects obesity, but associated with special symptoms: adiposis dolorosa. Am J Med Sci 104: 521-535
6. Hansson E et al. (2012) Review of Dercum's disease and proposal of diagnostic criteria, diagnostic
   methods, classification and management. Orphanet J Rare Dis 7: 23.
7. 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
8. Steiner J (2002) Lipomatosis dolorosa--a frequently overlooked disease picture. Neurologist 73: 183-187
9. Wollina U et al. (2018) Differential diagnosis of lipedema and lymphedema. Dermatologist 69:1039-1047