Adiposis dolorosa
Dr. Francis Xavier Dercum was a physician in the United States who headed the University of Pennsylvania’s Neurological Clinic and was the personal physician to President Wilson. Dercum first described in 1888(1) a group of signs and symptoms that now bear his name, Dercum’s disease (DD). Of note here is that Dercum’s disease is more of a syndrome than a disease, so I prefer the term adiposis dolorosa (AD) when writing about it; however, in casual conversation or by e-mail, I revert to using the abbreviation, DD. The reason for this is that I think adiposis dolorosa is a syndrome because it has several clinically recognizable features, signs, and symptoms that are characteristic of it and that tend to occur together, so that the presence of one feature, for example, painful fat, can alert a physician or patient to the presence of the others. More importantly than which name we use, however, is that we increase recognition of this syndrome by physicians and patients; I think the names adiposis dolorosa and Dercum’s disease will both stay in use.
Adiposis dolorosa is a syndrome of painful growths in subcutaneous fat. The fatty growths can occur anywhere in subcutaneous fat from scalp to plantar surface of the foot, are unencapsulated, soft, sometimes occurring as small nodules, and other times are difficult to remove in entirety because of their expansive growth(2). The onset of AD can be rapid or insidious and progressive beginning most often in the third decade(2) with fatigue(3, 4) and pain unresponsive to analgesics(5). Obesity is common in AD with some describing rapid weight gain(6). Some cases of AD are familial(5, 7, 8). Pain in AD is difficult to treat(5) and although surgical resection or liposuction improves pain, growths recur in 50%(2). The etiology of AD is unknown, although metabolic or autoimmune components have been proposed(2, 5).
In a survey of 110 individuals with AD, 16.4% had diabetes(2). The percent of respondents with diabetes by body mass index (BMI) was as follows: 7.6% <25 kg/m2, 4.8% from 25.0-29.9 kg/m2, 7.1% from 30.0-34.9 kg/m2 and 29.2% ³35 kg/m2. Twenty-nine respondents (26.4%) were diagnosed with the metabolic syndrome or insulin resistanceor met NCEP guidelines for metabolic syndrome. In the ages between 45-64y, the prevalence of diabetes was over twice that of the 2005 US population(9). Comparing rates of diabetes by BMI, the percent of individuals in our survey with diabetes in the lowest and highest BMI groups was greater than that of published data from NHANES(10) suggesting that growths in AD might confer insulin resistance even in the absence of obesity. Another suggestion of metabolic derangement in the growths is that 100% of individuals with AD state that they cannot lose weight form fatty growths.
References
1. Dercum, F. X. (1888) A subcutaneous connective-tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema. In University Medical Magazine Philadelphia. Vol. 1 pp. 140-150.
2. Herbst, K. L., and Asare-Bediako, S. (2007) Adiposis Dolorosa is More than Painful Fat. The Endocrinologist 17, 326-344
3. Palmer, E. D. (1981) Dercum's disease: adiposis dolorosa. Am Fam Physician 24, 155-157
4. Harris, K., Davies, K., Dumont, S., and Stephenson, B. M. (1997) A pain in the groin. Lancet 350, 334
5. Wortham, N. C., and Tomlinson, I. P. (2005) Dercum's disease. Skinmed. 4, 157-162; quiz 163-1644.
6. Brorson, H., and Fagher, B. (1996) [Dercum's disease. Fatty tissue rheumatism caused by immune defense reaction?]. Lakartidningen 93, 1430, 1433-1436
7. Campen, R., Mankin, H., Louis, D. N., Hirano, M., and Maccollin, M. (2001) Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 44, 132-136
8. Cantu, J. M., Ruiz-Barquin, E., Jimenez, M., Castillo, L., and Macotela-Ruiz, E. (1973) Autosomal dominant inheritance in adiposis dolorosa (Dercum's disease). Humangenetik 18, 89-91
9. (2005) National Diabetes Fact Sheet. In, National Center for Chronic Disease Prevention and Health Promotion
10. Gregg, E. W., Cadwell, B. L., Cheng, Y. J., Cowie, C. C., Williams, D. E., Geiss, L., Engelgau, M. M., and Vinicor, F. (2004) Trends in the prevalence and ratio of diagnosed to undiagnosed diabetes according to obesity levels in the U.S. Diabetes Care. 27, 2806-2812.
Adiposis dolorosa is a syndrome of painful growths in subcutaneous fat. The fatty growths can occur anywhere in subcutaneous fat from scalp to plantar surface of the foot, are unencapsulated, soft, sometimes occurring as small nodules, and other times are difficult to remove in entirety because of their expansive growth(2). The onset of AD can be rapid or insidious and progressive beginning most often in the third decade(2) with fatigue(3, 4) and pain unresponsive to analgesics(5). Obesity is common in AD with some describing rapid weight gain(6). Some cases of AD are familial(5, 7, 8). Pain in AD is difficult to treat(5) and although surgical resection or liposuction improves pain, growths recur in 50%(2). The etiology of AD is unknown, although metabolic or autoimmune components have been proposed(2, 5).
In a survey of 110 individuals with AD, 16.4% had diabetes(2). The percent of respondents with diabetes by body mass index (BMI) was as follows: 7.6% <25 kg/m2, 4.8% from 25.0-29.9 kg/m2, 7.1% from 30.0-34.9 kg/m2 and 29.2% ³35 kg/m2. Twenty-nine respondents (26.4%) were diagnosed with the metabolic syndrome or insulin resistanceor met NCEP guidelines for metabolic syndrome. In the ages between 45-64y, the prevalence of diabetes was over twice that of the 2005 US population(9). Comparing rates of diabetes by BMI, the percent of individuals in our survey with diabetes in the lowest and highest BMI groups was greater than that of published data from NHANES(10) suggesting that growths in AD might confer insulin resistance even in the absence of obesity. Another suggestion of metabolic derangement in the growths is that 100% of individuals with AD state that they cannot lose weight form fatty growths.
References
1. Dercum, F. X. (1888) A subcutaneous connective-tissue dystrophy of the arms and back, associated with symptoms resembling myxoedema. In University Medical Magazine Philadelphia. Vol. 1 pp. 140-150.
2. Herbst, K. L., and Asare-Bediako, S. (2007) Adiposis Dolorosa is More than Painful Fat. The Endocrinologist 17, 326-344
3. Palmer, E. D. (1981) Dercum's disease: adiposis dolorosa. Am Fam Physician 24, 155-157
4. Harris, K., Davies, K., Dumont, S., and Stephenson, B. M. (1997) A pain in the groin. Lancet 350, 334
5. Wortham, N. C., and Tomlinson, I. P. (2005) Dercum's disease. Skinmed. 4, 157-162; quiz 163-1644.
6. Brorson, H., and Fagher, B. (1996) [Dercum's disease. Fatty tissue rheumatism caused by immune defense reaction?]. Lakartidningen 93, 1430, 1433-1436
7. Campen, R., Mankin, H., Louis, D. N., Hirano, M., and Maccollin, M. (2001) Familial occurrence of adiposis dolorosa. J Am Acad Dermatol 44, 132-136
8. Cantu, J. M., Ruiz-Barquin, E., Jimenez, M., Castillo, L., and Macotela-Ruiz, E. (1973) Autosomal dominant inheritance in adiposis dolorosa (Dercum's disease). Humangenetik 18, 89-91
9. (2005) National Diabetes Fact Sheet. In, National Center for Chronic Disease Prevention and Health Promotion
10. Gregg, E. W., Cadwell, B. L., Cheng, Y. J., Cowie, C. C., Williams, D. E., Geiss, L., Engelgau, M. M., and Vinicor, F. (2004) Trends in the prevalence and ratio of diagnosed to undiagnosed diabetes according to obesity levels in the U.S. Diabetes Care. 27, 2806-2812.
