Lymphangiectases and Lymphangiomata

Wegner,1in 1877, proposed the first histologic classification of lymphangiomata. It sufficed until 1921, when Fabry and Ziegenbein2reported 2 cases entitled ``lymphangiokeratoma circumscriptum naeviforme.'' These apparently differed from the condition designated lymphangioma circumscriptum by Wegner and simple lymphangioma by Watson3and Allen4in that they appeared later in life and revealed hyperkeratosis on histologic examination. In 1895, however, Kaposi5had reported congenital lymphangiomata which showed histologic features indistinguishable from either the case reported here or those of Fabry and Ziegenbein. Kaposi's cases clearly indicated that congenital lymphangiomata are occasionally covered by a thickened stratum corneum. The modern classification of Allen provides 2 convenient categories in which to place such lesions: (1) lymphangiectases (not congenital), and (2) simple lymphangioma (usually congenital). Allen also described the presence of thick-walled vesicles in areas of lymphedema, a finding known to occur in some patients several years after undergoing