Localized lymphedema (elephantiasis): a case series and review of the literature

Abstract

Background: Lymphedema typically affects a whole limb. Rarely, lymphedema can present as a circumscribed plaque or an isolated skin tumor. Objective: To describe the clinical and pathologic characteristics and etiologic factors of localized lymphedema. Methods: Case–control study of skin biopsy and excision specimens histologically diagnosed with lymphedema and presenting as a localized skin tumor identified during a 4‐year period. Results: We identified 24 cases of localized lymphedema presenting as solitary large polyps (11), solid or papillomatous plaques (7), pendulous swellings (4), or tumors mimicking sarcoma (2). Patients were 18 females and 6 males with a mean age of 41 years (range 16–74). Anogenital involvement was most frequent (75%) – mostly vulva (58%), followed by eyelid (13%), thigh (8%) and breast (4%). Causative factors included injury due to trauma, surgery or childbirth (54%), chronic inflammatory disease (rosacea, Crohn’s disease) (8%), and bacterial cellulitis (12%). Eighty‐five percent of these patients were either overweight (50%) or obese (35%). Compared with a series of 80 patients with diffuse lymphedema, localized lymphedema patients were significantly younger (41 vs. 62 years old, p = 0.0001), had no history of cancer treatment (0% vs. 18%, p = 0.03), and had an injury to the affected site (54% vs. 6%, p = 0.0001). Histologically, all cases exhibited dermal edema, fibroplasia, dilated lymphatic vessels, uniformly distributed stromal cells and varying degrees of papillated epidermal hyperplasia, inflammatory infiltrates and hyperkeratosis. Tumor size significantly and positively correlated with history of cellulitis, obesity, dense inflammatory infiltrates containing abundant plasma cells, and lymphoid follicles (p < 0.05). A history of cellulitis, morbid obesity, lymphoid follicles and follicular cysts predicted recurrent or progressive swelling despite excision (p < 0.05). Conclusions: Localized lymphedema should be considered in the etiology of skin tumors when assessing a polyp, plaque, swelling or mass showing dermal edema, fibrosis and dilated lymphatics on biopsy. A combination of lymph stasis promoting factors (trauma, obesity, infection and/or inflammatory disorders) produces localized elephantiasis.

Giant angiofibromas in tuberous sclerosis complex: A possible role for localized lymphedema in their pathogenesis

“…Lu et al 37 identified histopathologic features of localized lymphedema in a variety of conditions, with variable factors including trauma, surgery, chronic inflammatory disease, and bacterial cellulitis. Paul and The same group has recently posited that localized lymphedema is an essential pathogenic factor in verruciform xanthomas.…”

Vulvar non-filarial elephantiasis associated with scabies

“…In its earliest stages, lymphedema is soft and pitting; however, with persistence, lymphedema has a brawny, non-pitting appearance likened to peau d'orange because of accentuation of skin folds and follicular ostia. Most of the swelling occurs in the subcutaneous tissue, but the skin exhibits the most changes, which, when severe, is termed elephantiasis [1]. Elephantiasis described a gross enlargement of the part of the body that has been involved.…”

“…Lu et al [1] presented a clinical series of 24 cases of localised lymphedema; he made a comparison between those patients and the patients with diffuse lymphedema. Lu found that the most frequent localisation in these patients is anogenital, and overall, women were more frequently affected by lymphedema, particularly in the anogenital region and trunk, than men.…”

“…Elephantiasis describes a massive enlargement of a limb or the external genitalia because of chronic lymphedema [1]. The elephantiasis of the vulva is a rare condition often caused by filariasis in developing nations [3].…”

Vulvar Lymphoedematous Pseudotumours Mistaken for Aggressive Angiomyxoma: Report of Two Cases

“…Nevertheless, a chronic localised lymphoedema presenting as a neoplastic mass is a rare condition. Lu et al [2] reported a series of 24 cases of localised lymphoedema (elephantiasis) presenting as solitary large polyps, plaques and pendulous swellings, some mimicking a soft tissue tumour. In this series, anogenital involvement was most frequent, particularly in the vulvar region.…”