Abstract:

Massive localized lymphedema (MLL) is a rare and underdiagnosed benign tumefactive pseudosarcomatous lesion, often associated with morbid obesity. Other predisposing factors include: trauma, previous surgery and hypothyroidism. MLL, typically presents as a large mass involving the thigh or abdomen, with an average specimen weight in excess of 8000 g. However, a subset of MLL can also manifest with small cutaneous polypoid or verrucous lesions that clinically mimic a variety of entities including condyloma, angiomyxoma, or angiofibroma. Notably, these cutaneous lesions can also arise in the absence of MLL, albeit rare. The verrucous cutaneous manifestations of MLL have historically been described under a variety of names including fibrous edema consistent with MLL, verrucous localized lymphedema of genital areas, and localized vulvar lymphedema, thus creating confusion, and a lack of nosologic consistency. Herein, we present three cases of MLL with these cutaneous findings. The lesions were located on the thigh and genital regions of both men and women, with associated poor aesthetics and disruption of daily activities. Clinically, they show varying degrees of confluent verrucous hyperplasia and papillomatosis of the epidermis. The histopathological findings are all similar, and bear a striking resemblance to the features seen in MLL which includes: hyperkeratosis, an expanded and partially fibrotic dermis with alternating layers of edema and fibrosis, and irregularly shaped lymphatic vessels with small, thick-walled blood vessels present in the dermis and subcutis. Accordingly, we propose a unifying diagnostic term: cutaneous lymphedema pseudotumor (CLP) for these cutaneous lesions both in the presence or absence of MLL. We believe that this nosologic lexicum, best encapsulates the varied clinical appearance and consistent histopathologic phenotype seen in CLP.

Financial Disclosure:
No current or relevant financial relationships exist.

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Jesse Sheldon, DO