Lymphangioma-like Variant of Kaposi's Sarcoma: Clinicopathologic Study of Seven Cases with Review of the Literature

Abstract

The clinical and pathological features of seven cases of lymphangioma-like Kaposi's sarcoma (KS) are reported. As with the other subtypes of KS, the lymphangioma-like variant occurs more often in men aged 59-80 years. Clinically, the lesion appears intermingled with the classical forms of KS, but a "bulla-like" appearance recognized in seven of 13 cases has been considered as a clinical hallmark of this variant. Although occasional cases have shown aggressive behavior, the most frequent clinical course is slowly progressive with localized or diffuse involvement of lower limbs. The histological pattern, characterized by permeation of dermal collagen by labyrinthine vascular channels lined by a flattened endothelium, must be differentiated from spindle cell hemangioendothelioma, low-grade angiosarcoma, targetoid hemosiderotic hemangioma, and benign lymphangioendothelioma.

Histopathological analysis of vesicular and bullous lesions in Kaposi sarcoma

“…Dermal edema and enlarged lymphatic vessels are prominent in bullous KS cases, and fibrosis can be observed in those patients who receive radiotherapy [8-13]. In our study, hyperkeratosis was observed in all bullous KS cases, and ulceration was detected in two cases.…”

“…To date, 30 reported cases with different terminologies have been available (Table 3) [5-21]. Cossu et al reported bullous KS frequency at 4% in epidemic KS cases [13]. Notwithstanding, in our series composed of classic KS cases, vesiculobullous changes were observed in 14% of all patients, whereas only 2% of these met the diagnostic criteria of bullous KS.…”

“…Cossu et al reported that bullous changes are more frequent in KS cases showing lymphangioma-like features [13]. However, Cottoni et al advocate that bullous changes cannot be related to any specific histological subgroup [10].…”

Acquired aquagenic papulotranslucent acrokeratoderma

“…1 The following criteria were suggested: (1) uniformly tan cribriform macular pigmentation in a zosteriform distribution; (2) a histologic pattern that consisted of a mild increase in melanin pigment in the basal cell layer and complete absence of nevus cells; (3) no history of rash, injury, or inflammation to suggest postinflammatory hyperpigmentation; (4) onset well after birth with gradual extension -age at onset was in the second decade of life in every case; and (5) lack of other associated cutaneous or internal abnormalities. 1 The following criteria were suggested: (1) uniformly tan cribriform macular pigmentation in a zosteriform distribution; (2) a histologic pattern that consisted of a mild increase in melanin pigment in the basal cell layer and complete absence of nevus cells; (3) no history of rash, injury, or inflammation to suggest postinflammatory hyperpigmentation; (4) onset well after birth with gradual extension -age at onset was in the second decade of life in every case; and (5) lack of other associated cutaneous or internal abnormalities.…”

“…Occasionally, there are associated systemic abnormalities. 1,2 The bullous lesion has been considered as a variant of a KS plaque, while an association with alterations in lymphatic drainage 7 and chronic lymphoedema 5 has been suggested. The linear nature of the pigmented bands probably reflects the clonal migration and proliferation of embryonic melanocyte precursors (melanoblasts).…”