Table 1.

Differential Diagnosis for Elephantiasis Nostras Verrucosa

Primary (congenital) lymphedemaCongenital abnormality in formation or function of lymphatic vessels and has a morphology similar to that of ENV. Onset is usually before the age of 25 and there is often a family history of lymphedema.5, 6
FilariasisCaused by Wuchereria parasites blocking lymphatic vessels and causing secondary bacterial infections. Morphology similar to that of ENV. Either endemic to tropical regions or a travel history to these regions is present. Diagnosis is confirmed on blood smear, antigen testing, or identification of the parasite on tissue biopsy.2
LipedemaAn abnormal amount of subcutaneous fat, typically bilateral and symmetric, that spares the feet.“Stove pipe legs” with a negative Stemmer sign. Early age of onset, usually female, with a positive family history. Histologically has no epidermal changes.2
LipodermatosclerosisCaused by venous stasis and resulting in subcutaneous fibrosis proximally that results in an “inverted wine bottle” look to the affected leg.2
Papillomatosis cutis carcinoidesMorphology similar to that of ENV, typically presents with ulcers, and is considered a low-grade squamous cell carcinoma. Distinguished from ENV by biopsy.1, 2
Pretibial myxedema (hyperthyroidism, Grave disease)Caused by mucin deposition within the dermis, causing nonpitting edema with thickened, nodular plaques over anterior leg and dorsum of the foot. Patients will have other sequelae of hyperthyroidism (ie, exophthalmos) and will have thyroid function test abnormalities.2, 3
Stewart-Treves syndromeAngiosarcoma that develops in chronic lymphedema and presents as an erythematous mass. Seen in patients after radical mastectomy and in congenital lymphedema.2, 5
Venous stasis dermatitisPresents as a pitting edema with erythema, skin desquamation, and pruritis. Does not have a verrucous appearance.3
Deep venous thrombosisAcute onset of pitting edema, typically in lower extremities, with no epidermal changes.5
Papular mucinosisIdiopathic disease distinguished by acid glycosaminoglycan infiltration of the papillary dermis. Presents clinically as waxy papules that can coalesce into plaques.2
PodoconiosisLymphatic obstruction caused by chronic, barefoot walking on volcanic soils with high silica dust content.3
  • ENV, elephantiasis nostras verrucosa.