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Adiposis dolorosa
Adiposis dolorosa
Clinical features Adiposis dolorosa (Dercum disease) is a rare disease characterized by painful, circumscribed areas with increased fat in a plaque-like distribution.1โ3 Usually, multiple body sites are involved but there is predilection for the buttocks, lower limbs and abdomen, particularly in juxta-articular areas.
1704 Connective tissue tumors
Localized disease is very rare and the breasts may be involved.4,5 The condition is much more common in females, between the fourth and sixth decades of live, especially after the menopause, and patients often have associated obesity and psychological problems.1โ3 Occasional cases are inherited in an autosomal dominant manner.6,7 A case associated with the use of corticosteroids, a further patient with hypercholesterolemia and severe atherosclerosis and one with lipomatous hypertrophy of the interatrial septum have been documented.8โ10 Pain occurs mainly as the result of palpation but may also occur spontaneously or as a result of movement.
A new classification of the disease has been proposed: (1) generalized diffuse without lipomas; (2) generalizsed diffuse associated with lipomas; (3) localized nodular; and (4) juxtaarticular.11
Fig. 35.14 Piezogenic pedal papules: note the multiple flesh-colored papules on the heel and lateral border of the foot. From the collection of the late N.P. Smith, MD, The Institute of Dermatology, London, UK.
Fig. 35.15 Lipomatosis of nerve: note the extensive epineural fat deposition.