Are You Confident of the Diagnosis?
What you should be alert for in the history
Lesions are usually noted postpuberty, presenting as painful nodules.
Characteristic findings on physical examination
Small (<4 cm), slow-growing soft subcutaneous nodules are most commonly distributed on the trunk or extremities; The infiltrating type is much less common and is usually seen in individuals over 30 years of age and on the extremities and are identical in clinical presentation as a lipoma.
Expected results of diagnostic studies
Histopathology demonstrates normal-appearing mature adipose accumulation with increased vasculature and characteristic thrombi in the microvasculature (Figure 1, Figure 2). Computed tomography (CT) without contrast demonstrates homogenous low-density areas; CT with contrast reveals enhancement due to increased vasculature.
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Figure 1.
Angiolipoma. (X40) (Courtesy of Cloyce L. Stetson, MD)
Figure 2.
Angiolipoma. (X200) (Courtesy of Cloyce L. Stetson, MD)
Magnetic resonance imaging yields better tissue delineation and extent of tumor, especially if there is a concern for infiltrating types; findings include homogenous low intensity areas with no contrast enhancement and heterogenous areas with extremely high contrast enhancement.
Diagnosis confirmation
Lipomas are less likely to be associated with pain; histologically they are similar but not associated with increased vasculature. Epidermal inclusion cysts display an overlying punctum, are less stable in size, and histologically distinct. Pilar cysts are found usually on the scalp, presenting as marble-like nodules; they are histologically distinct;
Angioleiomyomas are histologically distinct as are subcutaneous neurofibromas, subcutaneous dermatofibromas, neurilemmomas, rheumatoid nodules (associated with rheumatologic disease), pseudorheumatoid nodule/ deep granulare annulare, subcutaneous sarcoidosis, abscesses, and hematomas (associated with trauma).
Who is at Risk for Developing this Disease?
Lesions are typically noted postpuberty, in young men in the second and third decades of life.
What is the Cause of the Disease?
Etiology
The etiology involves fat accumulation, with increased microvasculature and characteristic microthrombi.
Pathophysiology
The pathophysiology is unclear.
Systemic Implications and Complications
Adiposis dolorosa (Dercum disease) presents as multiple (hyperalgesia) painful nodules, more commonly in middle-aged, overweight women. Characteristically, lesions are located on the upper legs, trunk, and upper arms. Emotional and psychiatric problems are commonly associated. Patients may need to be referred for psychiatric treatment.
Angiolipomas can present in many different locations (some reports include thyroid, intraosseous, breast, gastrointestinal tract) and can mimic otherwise concerning conditions there but there is no evidence that cutaneous angiolipomas are related to their presence.
Treatment Options
The surgical option is excision. Mesotherapy has been described for treatment of lipoma(s.
Optimal Therapeutic Approach for this Disease
If treatment is desired, excision remains the “gold standard.” The infiltrating type has a reported recurrence rate of 50%; mesotherapy has been described for lipomas.
Patient Management
None, unless mental or psychiatric problems associated with adiposis dolorosa are suspected.
Unusual Clinical Scenarios to Consider in Patient Management
Dercum disease is difficult to treat, being associated with emotional or psychiatric problems
The infiltrating type is very likely to recur because it is so difficult to completely remove surgically.
What is the Evidence?
Bua, JA, Luaces, R, Franco, FL, Garcia-Rozado, A, Escudero, JLC, Capdevila, EF, Lopez-Cedrun, JL. “Angiolipoma in head and neck: report of two cases and review of the literature”. Int J Oral Maxillofac Surg. vol. 39. 2010. pp. 610-25. (Two case reports of a rare presentation of angiolipoma followed by review of angiolipomas.)
Pandya, KA, Radke, F. “Benign Skin Lesions: Lipomas, Epidermal Inclusion Cysts, Muscle and Nerve Biopsies”. Surg Clin N Am. vol. 89. 2009. pp. 677-87. (Review of lipoma and epidermal inclusion cysts, including work-up and treatment.)
Naversen, DN, Trask, DM, Watson, FH, Burket, JM. “Painful tumors of the skin: "LEND AN EGG"”. J Am Acad Dermatol. vol. 28. 1993. pp. 298-300. (Case report of a painful granular tumor and review of painful cutaneous tumors.)
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. vol. 29. 2008. pp. 17-22. (Case report of an alternative treatment for lipomatosis dolorosa and review of this entity.)
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