Patient with multiple painful subcutaneous nodules

CORRECT DIAGNOSIS:

Adiposis Dolorosa (Decrum’s Disease)

DISCUSSION:

Adiposis dolorosa mainly affects women, especially those who are obese and postmenopausal, with ages ranging from 35-50 [1]. This condition presents with multiple painful lipomas of the arms and trunk. Pain can be severe, debilitating, and come in periodic waves. The pain is thought to be caused by compression of nerves by the lipomas. The lipomas vary in size and shape. Co-morbidities may include fibromyalgia, hypothyroidism, and diabetes. Other symptoms may consist of anxiety, memory issues, fatigue, sleep disorders, depression, and difficulty breathing [2].
On histopathology, the findings are usually consistent with a general lipoma, but may also show other pathology such as an angiolipoma [1]. Lab work is usually negative for inflammatory or autoimmune markers [2].
A review from 2012 suggests a four part classification [1]:
• Type I: Generalized diffuse form, with painful adipose tissue without lipoma formation
• Type II: Generalized nodular form, with pain in adipose as well as in and around lipomas
• Type III: Localized nodular form, with pain in and around lipomas
• Type IV: Juxtaarticular form, with excess fat deposits around joints such as the knee
The patient we are presenting in this case most likely has type II: generalized nodular form. Treatment is targeted to control pain. Multiple treatments have been proposed, but there is not a single treatment proven to be fully effective. The treatment approach should include multidisciplinary teams in order to assist with pain management. Starting with basic NSAIDs for pain control is prudent, but can be ineffective. Some case reports list these medications as treatment: metformin [3], infliximab and methotrexate [4], interferon alpha-2b [5] and pregabalin [6]. In the case of interferon alpha-2b, the patient did have a history of hepatitis C. Liposuction is also an option [7] as is surgical excision, but unfortunately the lipomas can reoccur.

TREATMENT:

Our patient’s goal for treatment was to decrease irritation and pain from the angiolipomas. Since pregabalin and gabapentin were not controlling the patient’s pain completely, dronabinol 2.5mg was tried twice daily for four months. At the return visit, the patient revealed that dronabinol did not alleviate the pain, and at this visit, she also requested surgical excision treatment. We recommended off-label use of apremilast for its anti-inflammatory properties. Samples were provided for the patient at this visit, as well as a referral to plastic surgery for surgical excision of some angiolipomas. Selenium 600-800mg once daily, cimetidine 400mg BID, and Excipial™ bruising pills were also recommended due to additional anti-inflammatory properties. At the next return visit, the patient stated she did not tolerate apremilast secondary to headaches. She was started on amitriptyline 25 mg twice daily, and 0.5 mg of naltrexone once daily for one week and increasing the dose by 0.5 mg weekly until at a dose of 3 mg for more adequate pain control. The patient was seen three months later and stated her pain was well controlled at this time. She had seen plastic surgery where she had 30 angiolipomas excised from her bilateral extremities. Current pain control regimen includes naltrexone 3 mg once daily, amitriptyline 25 mg as needed and adding gabapentin 300 mg three times daily. Although she did report dizziness with gabapentin use in the past, pregabalin became too expensive. She is due for follow-up in three months.

REFERENCES:

[1] Hausson E, Sevnsson H, Brorson H. Review of Dercum’s disease and proposal of diagnostic criteria, diagnostic methods, classification and management. Orphanet J Rare Dis. 2012;7:23–38. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3444313/

[2] Herbst KL, Asare-Bediako S. Adiposis Dolorosa is More than Painful Fat. The Endocrinologist 2007;17:326-44.

[3] Labuzek K, Liber S, Suchy D, Okopiea BA. A successful case of pain management using metformin in a patient with adiposis dolorosa. Int J Clin Pharmacol Ther 2013;51:517-24. doi: 10.5414/CP201878.

[4] Singal A, Janiga J, Bossenbroek N, Lim H. Dercum’s disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate. J Eur Acad Dermatol Venereol 2007;21:717. 16

[5] Gonciarz Z, Mazur W, Hartleb J, et al. Interferon alfa-2b induced long-term relief of pain in two patients with adiposis dolorosa and chronic hepatitis C. J Hepatol 1997;27:1141.

[6] Lange U, Oelzner P, Uhlemann C. Dercum’s disease (Lipomatosis dolorosa): successful therapy with pregabalin and manual lymphatic drainage and a current overview. Rheumatol Int. 2008;29:17–22. doi: 10.1007/s00296-008-0635-3.

[7] Wollina U, Goldman A, Heinig B. Microcannular tumescent liposuction in advanced lipedema and Dercum’s disease. G;145:151-9. http://lipedemaproject.org/wp-content/uploads/2016/02/2010_Wollina_Microcannular-Tumescent-Liposuction-in-Advanced-Lipedema-and-Dercums-Disease.pdf.