A rare condition that affects the skin above the ankles, lipodermatosclerosis refers to a skin change of the lower legs that often occurs in patients who have venous insufficiency. It is a type of panniculitis (inflammation of subcutaneous fat). Two-thirds of affected patients are obese.It tends to occur in people with chronic vein problems, which affects the blood flow in this area of skin.
Affected legs typically have the following characteristics:
-Smooth looking skin
-Increased pigmentation (brown skin)
-“Inverted champagne bottle” or “bowling pin” appearance.
Lipodermatosclerosis has also been called hypodermitissclerodermiformis, and sclerosingpanniculitis. The lower inner leg of one or both legs may be involved. It may present as an acute, or chronic condition.
It generally occurs without any preceding illness or local injury. Also, it presents as episodes of painful inflammation in the inner leg above the ankle, and resembles cellulitis. The affected area is red, tender and warm, and may be scaly. Some thickening of the skin can be felt but this is not sharply demarcated as in the chronic stage. Patients with acute lipodermatosclerosis are mainly middle-aged.
It may follow an acute episode or develop gradually. Common findings in chronic lipodermatosclerosis include:
-Hardening of the skin
-Small white scarred areas (atrophie blanche)
-Increased fluid in the leg (edema)
At this chronic stage, you are predisposed as well to venous or stasis eczema.
What causes it?
The cause of lipodermatosclerosis is not well understood. It appears to relate to venous hypertension (raised pressure in the leg veins), venous incompetence (leaky valves), and obesity.
It has been proposed that there is an acute inflammatory stage of lipodermatosclerosis, which is followed in several months or perhaps even years by a chronic stage. This is characterised by extensive fibrosis or sclerosis (scarring) in the skin and subcutaneous tissue. However, not all patients with chronic lipodermatosclerosis recall an acute stage.
The acute stage can occur before there are obvious signs of venous disease. Back pressure in the capillaries results in the activation of cells and soluble factors which encourage inflammation. In contrast, two-thirds of those with the late fibrotic phase, have obvious venous incompetence and venous hypertension. Subtle changes in blood clotting may also be a factor.
It is usually diagnosed clinically, but a skin biopsy is not done routinely. Dermatopathology shows histological changes mainly affect the fat, and depend on the stage of the disease.
Early lesions tend to show an infiltration of lymphocytes (a type of white cell), and areas of tissue death in the fibrous tissue dividing the fat. For intermediate lesions there is a mixed infiltrate of white cells and new fibrous tissue in the septa. Fibrous zones are present in the fat.
Late lesions reflect marked fibrosis in the fat, with diminished or absent inflammatory cells. Changes in the dermis include a mixed inflammatory cell infiltrate, increased fibrous cells, atrophy, or both, and tortuous thick-walled veins. Fibrin cuffs are present around the capillaries by direct immunofluorescence.
The histology stages correlate well with the two stages seen clinically. Blood tests are not usually required in lipodermatosclerosis but coagulation may be tested. Ultrasound scans and magnetic resonance imaging may be used to define the extent of the disease and to determine whether there is a role for vascular surgery.
Management of lipodermatosclerosis
The most important part of management is compression therapy to correct venous stasis. Seek the advice and care of your podiatrist and lymphedema therapist. Management may also include:
-Vein surgery, endovenous laser ablation, or sclerotherapy
-Medication to increase blood flow
-Steroid injections to reduce inflammation
-Medication to reduce pain