Dercum’s Disease (Lipomatosis Dolorosa) – a Rarely Diagnosed Disease: a Case Study

Česká verze

Authors: J. Hržičová ¹; S. Jančo ¹; B. Kollár ¹; F. Ondriaš ²; P. Turčáni P ¹
Authors place of work: I. neurologická klinika LF UK a FNsP, Bratislava ¹; Oddelenie patológie, FNsP Bratislava, pracovisko Ružinov ²
Published in the journal: Cesk Slov Neurol N 2007; 70/103(6): 707-709
Category: Kazuistika

Summary

Dercum’s disease is a rare condition characterized by development of painful subcutaneous fatty tissue deposits. The etiology remains unknown. Genetic factors, autoimmune mechanisms, microcirculation disturbances as well as alteration in lipid and carbohydrate metabolism are under the consideration. Typically postmenopausal obese women in age 35–50 years are affected. The most characteristic symptom is generalized or local pain of subcutaneous fatty tissue deposits often associated with asthenia, weakness, fatigability and mental disturbances, including emotional instability, depression, confusion and dementia. Diagnosis is made purely clinically and therapy is only symptomatic. To improve quality of life of affected individual combination of medications, surgery and psychiatric care is necessary. Presented case report should draw attention to this rarely diagnosed clinical condition.

Key words:
Dercum’s disease – lipomatosis dolorosa

Zdroje

1. Brorson H, Fagher B. Dercum's disease. Fatty tissue rheumatism caused by immune defense reaction? Lakartidningen 1996; 93: 1433-1436.

2. Dercum FX. Three cases of hitherto unclassified affliction resembling in its grosser aspects obesity, but associated with special nervous symptoms – Adiposis dolorosa. Am J Med Sci 1892; 104: 521-535.

3. Campen R, Mankin H, Louis DN, Hirano M, Maccollin M. Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 2001; 44: 132-136.

4. Wortham NC, Tomlinson IP. Dercum‘s disease. Skinmed 2005; 4: 157-162.

5. Berntorp E, Berntorp K, Brorson H, Frick K. Liposuction in Dercum's disease: impact on haemostatic factors associated with cardiovascular disease and insulin sensitivity. J Intern Med 1998; 243: 197-201.

6. Amine B, Leguilchard F, Benhamou CL. Dercums disease (adiposis dolorosa): a new case report. Joint Bone Spine 2004; 71: 147-149.

7. Brodovsky S, Westreich M, Leibowitz A, Schwartz Y. Adiposis dolorosa (Dercum's disease): 10-year follow-up. Ann Plast Surg 1994; 33: 664-668.

8. Chen SG, Hsu SD, Chen TM, Wang HJ. Painful fat syndrome in male patient. Br J Plast Surg 2004; 57: 282-286.

9. Steiner J, Schiltz K, Heidenreich, Weissenbom K. Lipomatosis dolorosa –ein häufig übersehenes Krankheitsbild. Nevenarzt 2002; 73: 183-187.

10. Singal A, Janiga JJ, Bossenbroek NM, Lim HW. Dercum's disease (adiposis dolorosa): a report of improvement with infliximab and methotrexate J Eur Acad Dermatol Venereol 2007; 21: 00-0 http//www.blackwell-synergy. com/doi/abs/10.1111/j.1468-3083.2006.02021.x