Localized Xanthomas in Lymphedema Praecox

Abstract

A girl, aged 14, had an extensive normolipemic xanthomatous eruption develop in one leg and foot, soon after the onset of lymphedema in the same limb. When the edema was reduced by treatment, the xanthomatous eruption greatly improved. It was speculated whether mechanical stretching of the skin might have been a factor contributing to the localized development of xanthomatosis in a xanthoma-prone individual.WE HAVE recently had the opportunity of observ¬ ing a girl in whom a striking number of xanthomatous nodules developed in one leg and foot, as the limb became lymphedematous. When the lymphedema was brought under control by physical measures, the xanthomas became smalle/. The obvious changes which had occurred during the appearance and subsequent shrinkage of the xantbomas were, respectively, an increase and decrease in the circumference of the limb, suggesting that the mechanical stretching of the skin may have had pathogenetic significance. Report of a CaseA school girl, aged 14, was referred for examination be¬ cause of swelling and a nodular eruption of the left leg and foot of 16 months' duration. The lateral part of the left ankle had been accidentally kicked by another child, resulting in local ecchymosis and soreness but little swelling. X-ray ex¬ amination of the ankle had disclosed no fracture. Several days later, the parents observed that the limb seemed slight¬ ly larger than the other one. Seven months later, it in¬ creased in size rather rapidly and became greatly swollen during an all-day bus and plane trip from New England to Florida. There was no pain, feverishness, or discoloration of the limb. The swelling persisted. About six weeks later, lymphangiography was performed. Three weeks later, a pruritic rash appeared on the left leg. This consisted of numerous yellowish papules which increased in number. Diuretics were given, resulting in considerable diuresis, weight loss, and generalized weakness, but little effect on the size of the leg or on the eruption. If the nodules were scratched, a whitish or a blood-tinged fluid would exude for a short period of time. Three years previously, a few similar papules, said to have been xanthomas, had been surgically removed from the inside of the left leg for cosmetic pur-poses. There was then no swelling. No lesions recurred until after the lymphangiographic procedure.Physical examination disclosed the patient to be of small stature, bright, and alert. The eyes were wide-set. The tip of the nose curved toward the midline of the upper lip but there was no median raphe or other sign of harelip. The palate was normal. Although the voice was immature, some axillary and pubic hair was present, and the breasts showed early signs of enlargement. (The first menstrual period had oc¬ curred three months before examination.) Mild facial acne was present. The blood pressure was 106/66 mm Hg; the height, 153.7 cm (5 ft V2 inch) ; and the weight, 38.6 kg (85 lb). There was marked swelling of the left lower limb, ex¬ tending from just below the knee to the toes (Fig 1...

Localized Xanthomas Associated with Primary Lymphedema

“…Pathogenetically speaking, lymphedema is initially characterized by edema and excess tissue protein, but it soon evolves into chronic inflammation and fibrosis and eventually hyperkeratosis, verrucous‐like skin, and elephantiasis. Xanthomas associated with lymphedema have been described in the dermatologic literature since the 1960s (2,3) in the form of isolated case reports (4–7). We believe that our patient developed verruciform xanthomas in the setting of a primary lymphedema.…”

Verruciform Xanthoma in Association with Milroy Disease and Leaky Capillary Syndrome

“…Cutaneous verruciform xanthoma associated with lymphedematous extremities was first described in 1963 by both Cairns (7) and Coburn (8). Since then there have been nine such reports (9–17). However, the presence of verruciform xanthoma has never been reported in the setting of Milroy disease or leaky capillary syndrome.…”

Lipid Biology and Lymphatic Function: A Dynamic Interplay with Important Physiological and Pathological Consequences

“…There are some indications that such concept may also hold in humans. Indeed, isolated case reports of xanthomas in primary lymphedema patients have been described in the dermatologic literature since the 60's [36][37][38][39][40]. Cholesterol accumulation in tissues as a result of poor lymphatic function may also increase the adipocyte burden to store more lipids thereby increasing deposition of fat [41,42].…”