D2‐40 highlights lymphatic vessel proliferation of angiolymphoid hyperplasia with eosinophilia

Abstract

Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign, uncommon idiopathic condition, characterized by cutaneous papules or nodules, whose etiopathogenesis is still unclear. It has been considered an angioproliferating lesion (epithelioid hemangioma) since histologically it is marked by a proliferation of blood vessels, accompanied by an inflammatory infiltrate, consisting mainly of lymphocytes and eosinophils. We present a case of ALHE assessed immunohistochemically for D2-40-a new marker for lymphatic endothelial cells. A biopsy specimen obtained from the same anatomical area of a healthy individual served as a normal control. The ALHE specimen showed increased number of lymphatic vessels when stained for D2-40, whereas the endothelial cells lining blood vessels were negative. The specificity of D2-40 for lymphatic vessels was further substantiated by studying Factor VIII-related antigen expression in consecutive sections of both ALHE and the control specimen. A reverse pattern was appreciated-blood vessels showed Factor VIII positive labeling, whereas lymphatic endothelial cells remained unlabeled. We therefore assume that apart from the lymphocytic infiltrate in the lesion, the recognized lymphoid component in ALHE is due to lymphatic vessel proliferation as well. Hence, this condition may be considered as possibly derived from lymphatic endothelium.

Lymphangiogenesis and Inflammation—Looking for the “Missing Pieces” of the Puzzle

“…Indirect evidences of eosinophils involvement in lymphangiogenesis derived from few studies describing an increase of lymphatic vessel density in angiolymphoid hyperplasia with eosinophilia, a benign, uncommon idiopathic condition, characterized by cutaneous papules or nodules and an inflammatory infiltrate, consisting mainly of lymphocytes and eosinophils (Miteva et al 2009). Eosinophils express CD30 and its expression is upregulated in certain conditions (Berro et al 2004).…”

Epithelioid hemangioma in the oral mucosa: A case report

“…In view of clinical similarities that EH shares with other diseases, histopathological examination is essential for establishment of the diagnosis. In the present case, the diagnosis was based on the typical histopathological features described in other studies [1,2,3,6]. The morphological characteristics consisted of well-developed vessels and some immature vessels lined by aggregates of epithelioid endothelial cells associated with an exuberant component of inflammatory cells, particularly eosinophils and lymphocytes.…”

“…First described by Wells and Whimser in 1969 as angiolymphoid hyperplasia with eosinophilia, epithelioid hemangioma (EH) is an uncommon benign vasoproliferative neoplasm whose etiology and pathogenesis continue to be a matter of debate [1,2,3]. The term epithelioid hemangioma was proposed by Enzinger and Weiss in 1983 [1].…”

“…EH commonly affects young adults and manifests as red or brown multiple nodules or small papules in the intradermal or subcutaneous region of the head and neck [1,2,4,5,6]. Extracutaneous lesions located in muscles, bone, and salivary glands are uncommon, and lesions involving oral mucosa are rarely observed [1,4,6,7].…”

Diagnostically Challenging Epithelioid Vascular Tumors

“…3). The tumor cells are positive for vascular markers, including D2-40, but immunoreactivity for keratins and EMA may be seen 18 and is another potential diagnostic pitfall.…”