Lymphangioma-Like Kaposi’s Sarcoma Presenting as Gangrene

Abstract

Kaposi's sarcoma (KS) is a multicentric vascular neoplasm associated with the Kaposi's sarcoma-associated herpes virus (KSHV). KS can occur in immunocompromised patients as well as certain populations in Africa or in the Mediterranean. Less than 5% of KS cases can present with lymphangioma-like kaposi sarcoma (LLKS), which can occur in all KS variants. KS presents with characteristic skin lesions that appear as brown, red, blue, or purple plaques and nodules. The lesions are initially flat and if untreated will become raised. LLKS presents similarly to KS but is associated with severe lymphedema and soft tissue swelling as well as bulla-like vascular lesions. We present the case of an 85-year-old Lebanese, HIV negative, man who presented with a swollen and painful right lower extremity accompanied by necrotic lesions. Wound cultures were positive, and we began the work-up for secondarily infected gangrene. However, skin biopsy results revealed that he in fact had lymphangioma-like Kaposi sarcoma, which allowed us to shift our management. Advanced Kaposi's sarcoma can present similar to gangrene. It is important to recognize the typical skin lesions of KS and not to overlook Kaposi's sarcoma or LLKS within the differential.

Palmoplantar lymphangioma‐like Kaposi sarcoma

“…Die unteren Extremitäten scheinen bevorzugt betroffen zu sein [6,8]. Es existieren nur vier Berichte über LLKS, die sich klinisch ausschließlich mit purpurfarbenen Maculae manifestieren, einer davon mit generalisierten Läsionen und die anderen drei mit Maculae an den unteren Gliedmaßen [4,8,9]. Jedoch ist die makuläre Manifestation des LLKS mit einer palmoplantaren Verteilung selten.…”