Dermatologic Manifestations of Localized Lipodystrophy Clinical Presentation

Updated: May 28, 2019
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Lipodystrophia centrifugalis is defined by the appearance of cutaneous lesions surrounded by a peripheral erythematous border before age 3 years. They may progress centrifugally for as long as 10 years and tend to regress thereafter. Children who are affected are healthy.

Involutional lipoatrophy is characterized by one or a few circumscribed depressions of the skin affecting any part of the body in persons of any age, with or without a history of previous trauma, pressure, or previous injections in the area. [17] Clinically, it usually appears as a solitary, asymptomatic, well-demarcated, atrophic depression and often involves areas of antecedent intramuscular or intra-articular injections. [18, 19, 20] Overlying hypopigmentation and atrophy with morphealike changes may be noted with histology studies.

Panniculitis precedes the onset of lipoatrophy in the inflammatory subsets but may be asymptomatic clinically.

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Physical Examination

Lipodystrophia centrifugalis abdominalis infantilis, as originally described by Imamura et al [21] in 1971, presents as a round or oval depression in the skin, often in the groin or the axilla. [22] It is characterized by one or a few circumscribed depressions or indentations in the skin. The overlying skin is of normal texture and color except for the periphery, which may be erythematous and a little scaly. Lesions tend to spread centrifugally to adjacent areas, such as the chest and the abdomen, for several years. The face and the sacral area can also be involved. Regional enlarged lymph nodes can often be palpated. No other abnormal finding is present clinically.

The lesions are round, oval, bandlike, horizontal, annular, or semicircular in configuration. They are flesh colored and barely visible with normal overlying skin. The anterior parts of the thighs, the buttocks, or the lower parts of the limbs, including the ankles in some cases, as well as the arms, the neck, and the scalp may be involved, unilaterally or bilaterally. The lesions may be mildly tender at first. No associated finding and no preceding sign of inflammation are present.

Localized lipoatrophy may represent the late stage of panniculitis. Indurated, tender, erythematous, purplish or hyperpigmented subcutaneous nodules or plaques usually involving the extremities precede the onset of lipoatrophy at the same sites.

Fever and systemic signs may be present depending on the type of associated panniculitis.

Lipophagic panniculitis of childhood, first described in children and later in adults, [23] is characterized by its benign self-limited course. It presents with multiple, slightly tender, erythematous subcutaneous nodules and plaques, which precede the onset of lipoatrophy. The lesions mainly occur on the extremities. It may recur several times, and it may be associated with intermittent fever.

Localized lipoatrophy may be the only presenting feature in patients with an underlying or evolving connective-tissue disorder, such as lupus erythematosus, morphea profunda, dermatomyositis, [24] or overlap disease. In patients with lupus panniculitis, multiple, often painful subcutaneous nodules or plaques, with a predilection for the proximal extremities, may be observed in association with irregular, indurated depressions in the same areas. The overlying skin is often normal, or changes, such as atrophy, hyperkeratosis, telangiectasias, and/or ulceration, seen in discoid lupus erythematosus may be present. Arthralgias or Raynaud syndrome may be observed, but most patients show no evidence of severe systemic involvement, such as glomerulonephritis or neurologic complications.

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