Xanthelasma, Xanthoma tuberosum, Xanthoma tendinosum, Eruptive xanthoma, Xanthoma planum, Palmar xanthoma, Tuberoeruptive xanthoma, Lymphedema, Achilles tendon xanthomas, Cutaneous xanthomas, tumorous xanthomas, eruptive xanthoma, Cytology, granulomatous inflammation, histiocytic infiltration, hyperlipidemia, Langhans giant cell;macrophages, Hypercholesterolemia, Xanthelasma
This is yet another skin growth that can accompany lymphedema as a skin growth complication. These are usually small growths (lesions) that develop as a results of an altered lipid (fat) metabolism or as a result of local cell dysfunction. (1) They are benign, but can be problematic if irritated, pulled or damaged. For those of us with lymphedema any irritation to any type of skin growth can lead to constant a draining of lymphorrea.
They may also be a sign of a medical condition that involces an increase in blood lipids.
These conditions include:
Certain cancers Diabetes Hyperlipidemia Inherited metabolic disorders such as familial hypercholesterolemia Primary biliary cirrhosis
One common type called, Xanthelasma palpebra occurs on the eyelids and may actually occur without any underlying medication condition or is not necessarily associated with elevated cholesterol or lipids. The lesions are soft, velvety, yellow and can be flat papules or plaques.
While most are small, some may actually reach 3 inches in diameter and can appear anywhere on the body, but are most often seen on the elbows, joints, tendons, knees, hands, feet, or buttock. (3)
Though the lesions themselves are generally without any complication, 30% of affected people have involvement of the lining of the mouth, airways or eyes (mucosal surfaces). Warty plaques in the mouth are called verruciform xanthomas.
40% of affected people develop diabetes insipidus, a condition that results in inability to control water loss (resulting in continual thirst and excessive urine production). This is due to histiocyte overgrowth on the lining of the brain (meninges). They can also affect internal organs.
The granulomas are filled with a yellow-liquid, that is cholesterol-rich.
There are numerous types of xanthomas:
Xanthoma tuberosum Xanthoma tuberosum is characterized by xanthomas located over the joints. These are firm, painless, red-yellow nodules that develope around pressure points such as the knees, elbows, heels and buttocks. They can join together to form what is called multilobated masses. These lesions are associated with imcreased cholesterol level in the blood and increased LDL levels. (4)
Xanthoma tendinosum Xanthoma tendinosum (also known as “Tendinous xanthoma”) is clinically characterized by papules and nodules found in the tendons of the hands. Tendon Xanthoma are associated with Type II hyperlipidaemia, chronic biliary obstruction, and primary biliary cirrhosis. They are associated with severe hypercholesterolaemia and elevated LDL levels. (4)
Eruptive xanthomas Eruptive xanthoma (ILDS E78.220) is clinically characterized by small, yellowish-orange to reddish-brown papules that appear all over the body. These erupt as crops of small, red-yellow papules most commonly over the buttocks, shoulders, arms and legs. They may be tender or/and unusually itchy. These lesions are associated with hypertriglyceridaemia (increased triglyceride levels in blood) often in patients with diabetes mellitus (sugar diabetes). (4)
Xanthoma planum Xanthoma planum (ILDS D76.370), also known as “Plane xanthoma”, is clinically characterized by macules and plaques spread diffusely over large areas of the body.
Palmar xanthomas Palmar xanthoma is clinically characterized by yellowish plaques that involve the palms and flexural surfaces of the fingers.:531 Plane xanthomas are characterised by yellowish to orange, flat macules or slightly elevated plaques, often with a central white area which may be localised or generalised. They often arise in the skin folds, especially the palmar creases. They occur in hyperlipoproteinaemia type III and type IIA, and in association with biliary cirrhosis. The presence of palmar xanthomata, like the presence of tendinous xanthomata, is indicative of hypercholesterolaemia.
Tuberoeruptive xanthomas Tuberoeruptive xanthoma (ILDS E78.210) is clinically characterized by red papules and nodules that appear inflamed and tend to coalesce. Tuberous xanthomas are considered similar, and within the same disease spectrum as tuberoeruptive xanthomas. (2)
Plane xanthomas These are flat papulaes or patches that can occur anywhere on the body. Lesions on the creases of the palms are indicative of a specific pattern of increased lipids in blood called type III dysbetalipoproteinaemia May be associated with hyperlipidaemia and hypertriglyceridaemia. (4)
Lesions on the creases of the palms are indicative of a specific pattern of increased lipids in blood called type III dysbetalipoproteinaemia.
Diffuse plane xanthomatosis A rare form of histiocytosis and is associated with an abnormal antibody in the blood called a paraprotein. Lipid levels are normal.
About 50% will have a malignancy of the blood; usually multiple myeloma or leukaemia.
Presents with large flat reddish-yellow plaques over the face, neck, chest, buttocks and in skin folds (such as the armpits and groin).
The only complication assocaited with Xanthomas is that they can be/are disfiguring.
Treatment consists of treating any condition that causes increased blood lipids.
They can be surgically removed, but often comeback after the surgery. For those of us with lymphedema, you may want to consider having them removed to prevent any future irritation or infection. The problem for us as with any type of minor skin growth is that leaving them on the skin usually will present some type of problem in the future. Removal can be either through the use of toppical trichloroacetic acid, electrodesiccation, laser vaporisation or a simple excision.
Treating the causes may also involve changes in the dietary regime which includes minimising saturated fats, intake of refined sugars, and reducing weight or caloric intake and increasing exercise.
There also may be medications prescribed.
Statins (HMG CoA reductase inhibitors), such as simvastatin and atorvastatin, reduce cholesterol production by the liver, resulting in lower LDL cholesterol levels, increase HDL cholesterol and mildly reduce triglycerides. Treatment should be monitored by regular blood tests to check the lipid levels and ensure liver and muscle enzymes are normal, as statins sometimes cause abnormalities especially when prescribed in higher doses.
Fibrates, such as bezafibrate, may be added to reduce triglyceride production by the liver, lowering triglycerids and increasing HDL cholesterol. They may cause gastrointestinal side effects.
Ezetimibe may be added in high risk patients or if higher doses of statins are poorly tolerated. It reduces cholesterol absorption from the gut, lowering total and LDL cholesterol.
Nicotinic acid lowers cholesterol, LDL cholesterol and triglycerides, and increases HDL cholesterol. At therapeutic doses of at least one gram daily, it causes flushing. An analogue, acipimox, is better tolerated. Cholestyramine and colestipol are rarely used as they are not as effective as the medications listed above and they are poorly tolerated. (4)
The prognosis is excellent, especially with local excision.
Localized xanthomas associated with primary lymphedema. Jan. 2012
Romaní J, Luelmo J, Sáez A, Yébenes M, Sábat M, Fernández-Chico N, Pifarré M, García D, Muñoz C.
Department of Dermatology, Hospital Parc Taulí, Sabadell, Barcelona, Spain Department of Pathology, Hospital Parc Taulí, Sabadell, Barcelona, Spain.
Cutaneous xanthomas arising in chronic lymphedema are rare. We present a case of verruciform xanthoma involving the left foot and toes of a 10-year-old boy who had developed a primary lymphedema (lymphedema praecox) in the left lower extremity. Laboratory studies demonstrated a normal lipid profile.
Peculiar distribution of tumorous xanthomas in an adult case of erdheim-chester disease complicated by atopic dermatitis. May 2011
Murakami Y, Wataya-Kaneda M, Terao M, Azukizawa H, Murota H, Nakata Y, Katayama I.
Department of Dermatology, Osaka University Graduate School of Medicine, Osaka, Japan.
Erdheim-Chester disease · Hand-Schüller-Christian disease · Xanthoma · Atopic dermatitis · Macrophage · CD68 · CD163 · Thymus - and activation-regulated chemokine
Erdheim-Chester disease is a rare non-Langerhans form of histiocytosis with multiple organ involvement. Approximately 20% of patients have xanthoma-like lesions, usually on the eyelids. We report a case of Erdheim-Chester disease in a 32-year-old male who showed peculiar xanthomatous skin lesions and also had atopic dermatitis. His skin manifestations included ring-like yellowish tumors on his periorbital regions, rope necklace-like tumors on his neck, and spindle-shaped tumors on his right preauricular region and cubital fossas. He also had exophthalmos and diabetes insipidus. Chronic eczematous lesions were present on the flexor aspect of his extremities, and his serum eosinophil numbers and immunoglobulin E levels were elevated. A histological examination of his right neck tumor showed foamy macrophages and touton-type giant cells, which were positive for CD68 and CD163 and negative for S-100 and CD1a. We suggest that the complication of atopic dermatitis may have contributed to the uncommon clinical features in this case.
Full Page Article:Karger]]
Nodular excision for painful localized Achilles tendon xanthomas in type II hyperlipoproteinemia: a case report. Sept.)ct 2911
Ahn JH, Chun TJ, Lee S.
Department of Orthopaedic Surgery, The Catholic University of Korea, Seoul, Korea.
Keywords: ankle, cholesterol, foot, lipid, surgery, statin
Achilles tendon xanthomas are often associated with type II hyperlipoproteinemia, in which low-density lipoprotein derived from the circulation accumulates in the tendons. Sometimes coronary artery disease can jeopardize the life of the patient if the condition is neglected. We describe the case of bilateral painful Achilles tendon xanthomas in a heterozygous type II hyperlipoproteinemia family. Her symptoms were not alleviated despite anti-inflammatory medication and eccentric exercise for 6 months. She was treated with nodular excision of the xanthomas bilaterally and then with postoperative statins to avoid recurrence.
Unknown: Yellow plaques on the chest and arms present for more than 6 years in an 80-year-old male. Diffuse normolipemic plane xanthomas presenting in association with myelodysplastic syndrome. May 2011
ICD-9 272.2 Includes: Tubo-eruptive xanthoma, Xanthoma tuberosum