Human Immunodeficiency Virus–Negative–Associated Lymphangioma-like Kaposi's Sarcoma with Variable Clinical Presentations

Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare histologic variant of KS. Kaposi's sarcoma is also known as human herpesvirus type 8. The clinical presentation of the LLKS lesion is highly unusual and similar to that of classic KS but with multinodular vascular tumors and lymphedema. We present a 63-year-old native Haitian man with multiple slowly progressive exophytic ulcerated lesions covering more than 60% of his left lower extremity with no systemic involvement. Much confusion surrounded the clinical presentation of these wounds, and we postulated several differential diagnoses. Herein we describe the evaluation, clinical appearance, and progression of LLKS. Due to the rarity of LLKS, treating physicians need to be aware of the clinical presentation and diagnostic criteria of this variant. Despite being incurable, early diagnosis of LLKS can lead to long-term treatment options and a major reduction in symptoms.