What to know
- Which types are seen with each familial hyperlipidemia
- What lab is elevated (tryglycerides vs cholesterol vs both)
- Increased risk for coronary artery disease
- What can trigger or exacerbate xanthomas
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Tuberous and Tendinous xanthomas are seen in type Two and Three and both start with the letter T. Patients have increased risk for coronary artery disease.
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Eruptive xanthomas
- present in Type 1, 4 & 5 hyperlipidemia
- Clinical presentation
- Small erythematous-yellow papules
- Extensor surfaces (eg. knees)
- Koebner phenomenon
- Hypertriglyceridemia (>3000-4000)
- Associated with extremely high tryglicerid levels
- Diabetes, obesity and alcoholism are risk factors → affect liver processing of lipids
- Can lead to acute pancreatitis
- Certain drugs can precipitate eruptive xanthomas
- Isotretinoin
- Olanzapine
- Alcohol
- Estrogen replacement
- Protease inhibitors
- These medications stimulate the liver to produce excess tryglicerides
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Tuberous Xanthomas
- Type 2&3 hyperlipidemia (Two and Three → Tuberous)
- Elevated cholesterol and tryglycerides
- Pink-yellow nodules over elbows and knees
- Seen in dysbetalipoproteinemia (with plane xanthomas of the palmar crease)
- Slow to regress
- Continuum with tuboeruptive xanthomas (which are smaller and usually interdigital)
- Xanthoma striatum palmare
- Xanthomas in the palmar creases
- In 2/3 of patients with type 3 hyperlipidemia
- Pathognomonic for type 3 hyperlipidemia
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Tendinous Xanthomas
- Over achilles and hand tendons
- Overlying skin is normal
- Dermal accomulation of lipids
- Most commonly seen in familial hypercholestrolemia type 2 and 3
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Plane Xanthomas
- Large, thin, smooth plaques
- In adults, should be screened for hyperlipidemia
- If no hyperlipidemia found, should suspect underlying monoclonal gammopathy of proliferative disorders
- If intertriginous → homozygous familial hypercholestrolemia
- If palmar → dysbetalipoproteinemia
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Xanthelasma
- Yellowish plaques around the eyelids
- Only 50% with hyperlipidemia
- Higher risk of hyperlipidemia in younger patients and with strong family history
- Screening recommended for dyslipidemia and monoclonal dysglobulinemia
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Verruciform Xanthoma
- Oral mucosa, anogenital, periorificial
- No associated hyperlipidemia
- Whitish plaques
- Seen in
- Lymphedema
- Epidermolysis bullosa
- GVHD
- CHILD syndrome
- Epidermolysis Bullosa
- Pemphigus
- DLE
- (disorders that are scarring or disturb the epidermis a lot)
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Tratamento
- TCA
- Cirurgia - cortar com tesoura