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ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
ANSWER
The correct answer is eruptive xanthomata (choice “b”) caused by an accumulation of lipid-filled macrophages as a result of pathologic levels of serum triglyceride—a situation discussed more fully below.
Neurofibromatosis type I (choice “a”), also known as von Recklinghausen disease, can present with multiple intradermal nodules. However, it usually appears in the second or third decade of life, with lesions that are fixed and soft. Biopsy would have confirmed this diagnosis.
Diabetic dermopathy (choice “c”) manifests with atrophic patches on anterior tibial skin. The patches occasionally become superficially eroded but do not resemble this patient’s lesions at all.
Juvenile xanthogranuloma (choice “d”) usually presents on children as a solitary yellowish brown papule. It can resemble eruptive xanthomata histologically but not clinically.
DISCUSSION
Eruptive xanthomata (EX) are relatively common, manifesting rapidly as papules and nodules, most frequently in the setting of hypertriglyceridemia. The latter can be familial and may be worsened by poorly controlled diabetes. Persons with Fredrickson types I, IV, and V hyperlipidemia are especially prone to EX.
As might be expected, patients with EX are at risk for several associated morbidities, including acute pancreatitis (especially in childhood cases) and atherosclerotic vessel disease. EX have also been associated with hypothyroid states and nephrotic syndromes.
Elevations in cholesterol, with normal triglyceride levels, can be associated with several types of xanthoma, including plane xanthomas and xanthelasma. The latter, often benign, can manifest in a normolipemic patient as well (necessitating a problem-directed history, physical, and lipid check).
Biopsy is often required to confirm the diagnosis of EX. As in this case, it typically shows monotonous collections of lipid-laden macrophages. Frozen sections of EX can be successfully stained for lipids, but routine processing of specimens effectively removes any lipids, replacing them with paraffin.
TREATMENT
Treatment entails controlling lipids with medication (fenofibrate), diet, and exercise and getting diabetes under control, as indicated. It is also essential to assess for atherosclerotic vessel disease and rule out pancreatitis.
Within a month of institution of treatment, this patient’s lesions had all but disappeared. His serum amylase and lipase were within normal limits, and testing for atherosclerotic vessel disease was pending.
Click here for more DermaDiagnosis cases, including
• The Value of Certainty in Diagnosis
• A Purplish Rash on the Instep
• Hair Loss at a Very Young Age.
Although they are unaccompanied by any other symptoms, this man is understandably alarmed by the extensive lesions covering much of his body. They first appeared months ago but have become more numerous, larger, and more prominent with time. The patient’s history includes type 2 diabetes (often poorly controlled) and dyslipidemia, for which he takes fenofibrate. Several years ago, he experienced a similar skin outbreak, which resolved after the patient increased his exercise and gained better control of his blood glucose. The condition is striking. There are widespread bilateral collections of shallow intradermal papules, nodules, and plaques primarily on the extensor surfaces of the patient’s arms, legs, and thighs and the convex surfaces of his buttocks. Numbering into the hundreds, the lesions spare his palms, soles, face, and scalp. No abnormality of the periocular skin is appreciated. Moderately firm on palpation, the lesions range in size from 1 to 3 cm in diameter. In several locations, they are linearly configured. A 4-mm punch biopsy of one of them shows large numbers of foamy macrophages in the epidermis and upper dermis. Bloodwork reveals a triglyceride level of 3,850 mg/dL.
