ABSTRACT: Age-related changes in the lymph and blood vessels contribute to the development of elephantiasis nostrum verrucosa, lipodermatosclerosis, atrophie blanche, and leukocytoclastic vasculitis. The reasons behind the increased incidence of giant cell arteritis in elderly persons remain unclear. Pyogenic granulomas are not common in older adults. However, because amelanotic melanoma and Kaposi sarcoma can mimic these benign vascular lesions, consider biopsy for any friable lesion or bleeding papule or nodule that appears in an elderly person.
All elements of the skin are affected by age. In this 2-part article, I discuss how the blood vessels, the lymphatics and the ground substance—which surrounds these vessels—respond to age, and I show how the aging elements of the vasculature can engender a variety of pathological cutaneous conditions.
Here I focus on elephantiasis nostrum verrucosa, lipodermatosclerosis, atrophie blanche, pyogenic granuloma, giant cell arteritis, and leukocytoclastic vasculitis. In Part 1, I discussed solar purpura, benign pigmented purpura, cherry angiomas, venous lakes, varicose veins, and spider telangiectasia.
ELEPHANTIASIS NOSTRUM VERRUCOSA
Figure 1 – Elephantiasis nostrum verrucosa can involve the entire leg, as in this patient.
Figure 2 – The nodules on this man’s leg represent elephantiasis nostrum verrucosa.
Figure 3 – Elephantiasis nostrum verrucosa can manifest as a pebble-surfaced plaque, as in this patient.
Figure 4 – The skin of this woman with lipodermatosclerosis appears lichenified and reddish brown because of the deposition of hemosiderin from red blood cells.
Elephantiasis nostrum verrucosa (ENV) is a rare, progressively deformative lymphedematous disorder that is also called chronic lymphedema, elephant skin, lymphangitis recurrens elephantica, or “mossy leg.” The disease is characterized by pronounced nonpitting edema with generalized lichenification, hyperkeratotic papules and nodules, and verrucous cobblestonelike plaques. ENV can involve the entire leg (Figure 1), or it can manifest as a discrete nodule (Figure 2) or a flat or pebble-surfaced plaque (Figure 3).
Although ENV resembles the traditional “elephantiasis” caused by the helminthic Wuchereria species, it represents a distinct entity not associated with filarial disease. ENV and traditional elephantiasis are both marked by impaired lymphatic drainage, which results in excess accumulation of interstitial fluid. Untreated ENV eventuates in pain and immobility.
Initial management of ENV of the lower extremities consists of prophylactic anti-streptococcal antibiotics, compression, and elevation, followed by pneumatic pump compression. These treatments, however, are obviously not appropriate for all regions of the body. Extensive ENV is very difficult to manage. A mainstay of treatment is weight loss.
If medical therapy is not effective, surgical measures include lymphovenous and lymphatic anastomosis as well as lymphatic transplantation. Amputation of the extremity is a last resort.
Lipodermatosclerosis is a term that describes changes in the leg caused by capillary proliferation, fat necrosis, and fibrosis of skin and subcutaneous tissues; these changes give the leg the clinical appearance of an “apple core” or “inverted champagne bottle.” Lipodermatosclerosis is associated with vascular insufficiency and stasis dermatitis. The affected skin is indurated and firm to the touch; it also appears lichenified and reddish or brown because of the deposition of hemosiderin from red blood cells (Figure 4). The differential diagnosis includes other forms of mixed septal and lobular panniculitis, including factitial causes.
Histological examination reveals adipocytes of variable size in the subcutaneous fat, including microcysts with thickened, eosinophilic walls. Fibrosis and a mild lymphocytic infiltrate are also evident. Direct immunofluorescence studies of early and late lesions show dermal pericapillary fibrin deposits without other immunoreactants.
Lipodermatosclerosis has also been called hypodermitis sclerodermiformis and sclerosing panniculitis. Confusion about the nature, clinical course, and treatment of lipodermatosclerosis exists. Some believe that lipodermatosclerosis has an acute inflammatory phase and a chronic fibrotic stage; others consider it to be a panniculitis.1,2
Treatment of lipodermatosclerosis consists of compression therapy with either graded stockings or elastic bandages. The anabolic steroid stanozolol(Drug information on stanozolol) can soften the skin and ameliorate any associated pain.