Adiposis Dolorosa (Dercum Disease) Differential Diagnoses

Updated: Aug 25, 2021
  • Author: Laura F McGevna, MD; Chief Editor: Dirk M Elston, MD  more...
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DDx

Diagnostic Considerations

Differential diagnoses include fibromyalgia, panniculitis, endocrine disorders, primary psychiatric disorders, multiple symmetric lipomatosis (Madelung syndrome), multiple familial lipomatosis, Proteus syndrome, and benign adipose tissue tumors. [1]

Like fibromyalgia, the diagnosis of Dercum disease (adiposis dolorosa) is made clinically, and both diseases include symptoms of a number of associated diseases. However, in Dercum disease, a relationship exists between pain and body weight, with pain in the fatty tissue and obesity being fundamental criteria for the diagnosis. In addition, the pain is often more general and more severe than in fibromyalgia.

Panniculitis presents with painful inflammatory nodules of the subcutaneous fat; however, the findings of erythema, ulceration, and atrophic scarring, as well as history and location, differentiate it from Dercum disease. Biopsy is diagnostic. [1]

Cushing syndrome and hypothyroidism are endocrine disorders also in the differential. These two diseases present with weight gain and generalized pain that is not localized to subcutaneous tissue as is seen in Dercum disease. [1]

Depression can also present similarly to Dercum disease as chronic pain conditions are often associated with depression. [1]

Madelung syndrome, also known as multiple symmetrical lipomatosis or benign symmetrical lipomatosis, is characterized by numerous, symmetrically distributed, nontender, poorly circumscribed lipomas, mainly around the neck, in the suboccipital region, on the proximal extremities, and on the upper part of the trunk. It is an idiopathic disease that affects middle-aged, nonobese men who are alcoholics. Neurologic involvement, particularly peripheral neuropathy, is considered a constitutive manifestation of this disease. [37]

Familial multiple lipomatosis belongs to the multiple lipoma syndromes. It is transmitted in an autosomal dominant fashion and often becomes apparent by the third decade of life. Patients may have up to hundreds of slowly growing, usually asymptomatic, subcutaneous lipomas of various sizes in widespread distribution. Patients with familial multiple lipomatosis are distinguished from patients with Dercum disease by their lack of disabling pain. [37]

Proteus syndrome is characterized by lipomas, partial gigantism of the hands or the feet, hemihypertrophy, pigmented nevi, and other subcutaneous neoplasms (eg, hemangiomas, lymphangiomas, mesenchymomas).

Weber-Christian disease (nonspecific panniculitis), neurofibromatosis, Fröhlich syndrome, adenolipomatosis, lipodystrophia progressiva, Cushing syndrome, and osteoarthritis should be ruled out when evaluating patients with multiple subcutaneous tumors. Also, in a patient who is obese, myasthenia gravis should be considered.

A patient with increasingly painful nodules resembling adiposis dolorosa was found to have calciphylaxis, and it is noted that the diseases may present similarly. [38]

Other conditions to consider include MERRF syndrome with lipomatous lesions, neurofibromatosis type 1, and multiple endocrine neoplasia type 1. [28]

Differential Diagnoses