Elephantiasis nostras verrucosa (ENV) is a rare, disfiguring sequelae of secondary chronic lymphedema, predisposing the patient to recurrent skin infections and resulting in cobblestone-like, papulonodular warts overlying woody, edematous tissue. The term “elephantiasis” characterizes gross enlargement due to lymphatic vessel obstruction. Nostras, meaning “from our region,” was first used in 1934 by Castellani in order to distinguish ENV from elephantiasis tropica or filarial elephantiasis, both of which are caused by parasitic lymphatic obstruction.1 Verrucosa describes the hyperkeratotic, wart-like appearance of the skin.2
Unlike other forms of elephantiasis, ENV is not caused by primary infection. Any condition that leads to lymphatic damage may increase a patient’s susceptibility to ENV in the future. Predisposing risk factors include lymphatic fibrosis from malignancy, radiation, or scleroderma; lymphatic obstruction from congestive heart failure or obesity; or traumatic disruption of the lymphatic system from surgery or injury.3 In a retrospective study of 21 patients with ENV, all patients were obese, with 91% morbidly obese; concurrent chronic venous insufficiency was identified in 71%, and 86% relayed a history of cellulitis or soft tissue infection on initial presentation.4
Patients who progress from chronic, noninfectious lymphedema to ENV enter a cycle of recurrent dermatologic and soft tissue infections with resulting fibrosis. In the setting of lymphatic damage, protein-rich lymph stasis results, inducing a local inflammatory response. This increases the region’s susceptibility to infections such as cellulitis, erysipelas, and lymphangitis, resulting in increased dermal fibrosis and reactive tissue edema. The overlying skin is made more vulnerable to infection in this setting as well, developing hyperkeratotic lesions over time. This pathologic progression of immune system dysfunction and infection leads to the characteristic appearance of ENV: nonpitting edema with superimposed verrucous lesions.2
Diagnosis is clinical, based on characteristic history and physical examination findings. History should include a description of the time course of symptom progression, a thorough exploration of chronic medical conditions, inquiry about a family history of lymphatic pathologies, and a travel history in order to differentiate ENV from related conditions. ENV is commonly found in gravity-dependent areas of the body such as the lower extremities and typically presents bilaterally; it often first appears on the dorsal aspect of the foot and progresses proximally. A positive Stemmer sign, or the inability to lift the skin fold at the base of the second toe, is diagnostic for lymphedema.2 This finding is due to lymphedema-induced skin thickening and would not be present in similar conditions that do not have changes in the skin. The disease has more rarely been observed in the upper extremities, abdomen, buttocks, scrotum, and face.5,6 The wart-like lesions may be malodorous due to recurrent bacterial or fungal infection. The cause of secondary lymphedema that predispose the patient to ENV can be investigated further via skin biopsy, lymphangiography, lymphoscintigraphy, computed tomography, or magnetic resonance imaging.6 Histologic findings for ENV include enlarged lymphatic vessels, fibrotic dermal tissue with loss of dermal papillae, and a hyperkeratotic epidermis with acanthosis and papillomatosis.2
The differential diagnosis for ENV includes venous stasis dermatitis, deep venous thrombosis, filariasis, lipedema, lipodermatosclerosis, papillomatosis cutis carcinoides, pretibial myxedema, and chromoblastomycosis.2,6 Although the clinical presentation of ENV is characteristic, blood smear or similar test should be performed to rule out Wuchereria bancrofti parasite infection, and skin biopsy should be performed to definitively rule out other similar diseases, especially if history and physical examination findings are ambiguous.
Although there is no clearly defined standard of care for ENV, treating the underlying causes is central to management. Compression stockings, massage, and medical wraps can help improve the lymphostasis. Medical treatment includes diuretics, which may help limit tissue edema; topical or systemic antibiotics and antifungals for acute infection or prophylactic purposes; as well as emollients such as salicylic or acetic acid. Systemic retinoids have also been used to help limit epidermal proliferation and inflammation, although they should be used carefully considering the drug’s teratogenic properties. Surgical options such as debridement, lymphatic transplant or anastomosis, or in severe cases amputation are also possible treatment options.2,6,7
Prognosis is dependent on the progression of ENV and underlying disease severity. When the disease is diagnosed early and the patient has regular clinical follow-up, outcomes are better. Without treatment, bacterial and fungal infections of the skin and underlying tissue can persist and spread to the bone or lead to sepsis. Due to the disfiguring nature of the disorder, social and psychiatric comorbidities such as anxiety and depression may have a significant impact on patient life.8 As the prevalence of chronic diseases such as congestive heart failure and obesity increases, it is likely that the incidence of ENV will also increase. Therefore, prevention of these predisposing conditions is key to preventing ENV.2The patient in the case described above elected to treat the condition with compression stockings and topical salicylic acid emollients.
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