Dercum’s disease (DD) is a disorder of painful fat tissue whose Hallmark is the development of lipomas. Dercum’s disease types have been reported but simply state if the lipomas are larger, smaller, diffuse or around joints without any suggestion of pathology.(1-3)  More recently, Herbst divided Dercum’s disease into the following types:

• Obesity
• Lipedema
• Healing disorder
• Familial multiple lipomatosis
• Angiolipomatosis

Other names for Dercum’s disease include Morbus Dercums and adiposis dolorosa.  The latter name is not useful as in Germany adiposis dolorosa is a name used to describe lipedema. 

Dercum’s disease affects all systems to a varying degree depending on how robust the onset, other co-morbidities, length of time with the disease, lifestyle, and life stressors. Some signs and symptoms of DD include fatigue, brain fog, insomnia, cardiac arrhythmia most often tachycardia (palpitations), gastrointestinal distress often similar to irritable bowel syndrome, muscle weakness, tremor or jerking of muscles (myoclonus), joint pains, insulin resistance and diabetes, and hypothyroidism or other autoimmune disorders.(3, 5)

Findings in the skin and adipose tissue from people with Dercum’s disease:

• Macrophages are increased, sometimes surrounding fat lakes (fatty cysts)
• Increased lymphatic vessels
• A lymphohistiocytic infiltrate surrounding small arterioles in adipose tissue and in the dermis of the skin, resulting in destruction of the vessels over time
• Enlarged capillaries in the dermis that migrate through the epidermis to the skin surface
• Increased collagen or fibrosis at times progressing to what is consistent with scleroderma like tissue

There are four potential causes of DD that are important to understand in order to be able to treat patients appropriately:

1. There is dysfunction of the lymphatic system from slowing of pumping inducing lymphangiogenesis secondary to fluid accumulation and resultant hypoxia (new lymphatics leak), to clogging and enlargement of lymph nodes, to profound lymphedema.
2. There is immune dysfunction, either because immune cells are missing a component (enzyme/other?), mast cells are activated, or because the immune system is being directed towards inflammation and away from its usual surveillance.
3. Dysfunction of the blood vasculature leading to increased blood vessel growth (angiogenesis with the formation of leaky vessels) to the extent that angiolipomas may form.
4. Likely partial mitochondrial deficiency likely secondary to systemic dysfunction as described above. This is important as when stress is increased (emotional stress, illness, trauma, over-eating), symptoms of DD increase and profound deconditioning results.

It also may be that nerve dysfunction, such as a sodium channel alteration, leads to alterations in nerve and lymph vessel function, at the same time lowering the threshold for activation of nerve pain fibers.

One paper suggests that Dercum’s disease can develop after infection including Lyme disease, coccidiodomycosis and histoplasmosis.(6)  This type of Dercum’s disease would be consistent with the healing disorder type.

Complications to watch out for include early cardiovascular disease and diabetes.(7)

References

1. Giudiceandrea V. L’adiposis dolorosa (malattia di Dercum). Riv Patol Nerv Ment. 1900;V:289-304.

2. Roux J, Vitaut M. Maladie de Dercum (Adiposis dolorosa). Revue Neurol (Paris) 1901;9:881-8.

3. Brorson H, Fagher B. [Dercum’s disease. Fatty tissue rheumatism caused by immune defense reaction?]. Lakartidningen. 1996;93(15):1430, 3-6. PubMed PMID: 8667732.

4. Herbst KL. Subcutaneous Adipose Tissue Diseases: Dercum Disease, Lipedema, Familial Multiple Lipomatosis and Madelung Disease. In: Purnell J, Perreault L, editors. Endotext. Massachusetts: MDText.com; 2019.

5. Herbst KL, Asare-Bediako S. Adiposis Dolorosa is More than Painful Fat. The Endocrinologist. 2007;17(6):326-44.

6. Beltran K, Wadeea R, Herbst KL. Infections preceding the development of Dercum disease. Infectious Disease Cases. 2019. Epub Dec 19, 2019. doi: 10.1016/j.idcr.2019.e00682.

7. Beltran K, Herbst KL. Differentiating lipedema and Dercum’s disease. Int J Obes (Lond). 2017;41(2):240-5.