Lymphangioma‐like Kaposi's sarcoma: report of four cases and review

Abstract

EditorKaposi's sarcoma (KS) is characterized by a wide range of cutaneous manifestations, such as macules, plaques, papules and nodules. However, some variants are only rarely observed and reported. 'Lymphangioma-like' or 'bullous KS' is very uncommon. The clinical features were first reported in 1957 by Ronchese and Kern. 1 Since then, only 21 cases have been published. We report the clinical correlation of four rare cases of lymphangioma-like KS with bulla-like lesions and review the literature.The first patient was a 65-year-old man with an 8-year history of pain and pruritus on the upper and lower extremities who presented with bulla-like lesions on both arms. These lesions varied in size from 0.5 to 2 cm in diameter. The second patient was an 89-year-old indigenous woman with a 1-month history of pain and oedema who presented with multiple nearly confluent violaceous nodules on her legs. Nodules varied in size from 0.5 to 1 cm in diameter. She also presented two bulla-like lesions on her left ankle. The third patient was a 61-year-old man with a 1-year history of pain and oedema who presented with multiple confluent brownish nodules and plaques on the lower extremities. Nodules varied in size from 0.5 to 1.5 cm in diameter. In these three patients, no lymphadenomegaly was clinically detectable, results of blood tests were within normal limits, and HIV serology proved negative. The fourth patient was a 36-year-old woman with a 5-year history of AIDS who presented with multiple violaceous plaques and nodules on her legs, and macules on her soft palate. Seven months before this she had been diagnosed with KS on the basis of her clinical presentation and histopathological features. At that time her lower extremities were treated with external radiation. Two months later, she experienced a rapid onset of bulla-like lesions on her right leg ( fig. 1). She was treated with systemic chemotherapy with no apparent response. The lesions had a tense consistency and were easily friable. Her CD4 count was less than 10 cells/mL and the viral load was 100 000 copies/mL. The patient died about 2 weeks later. fig. 1 Multiple confluent nodules and bulla-like lesions on the right foot.

Lymphangioma-Like Kaposi’s Sarcoma Presenting as Gangrene

“…Bulla-like lesions are the most common clinical feature seen in LLKS. Also, LLKS usually presents in the lower extremities, as was the case in our patient [8]. …”

Acquired Cutaneous Lymphangiectasia With Mesothelial Cells Reflux in a Patient With Cirrhotic Ascites

“…Kaposi sarcoma may rarely simulate lymphangioma, showing grossly dilated channels devoid of erythrocytes lined by flattened bland endothelial cells with slender intraluminal papillary projections, but in this variant more typical features of Kaposi sarcoma are usually associated. 34,35 In our patient, the nature of intravascular atypical cells was unmasked by calretinin expression on immunohistochemical study. Calretinin (CALB29, calbindin 2) is a 29-kDa calciumbinding protein that belongs to calbindin family, mainly found in neuronal tissue.…”

Histological Subgroups in Classic Kaposi Sarcoma

“…Lenfanjiyom benzeri varyant, labirent benzeri anastomozlaflan lenfatik vasküler yap›lar ile karakterizedir 2 . Nadir görülen bir alt tip olup s›kl›¤› %5'den azd›r 6,[17][18][19] . Ramirez ve ark., bu histolojik alt tipin klasik KS olgular›nda daha s›k (yaklafl›k %75) gö-rüldü¤ünü bildirmifllerdir 10 .…”