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Requena, L; El-Shabrawi-Caelen, L; Walsh, SN; Segura, S; Ziemer, M; Hurt, MA; Sangueza, OP; Kutzner, H.
Intralymphatic histiocytosis. A clinicopathologic study of 16 cases.
Am J Dermatopathol. 2009; 31(2):140-151 Doi: 10.1097/DAD.0b013e3181986cc2 [Oral Communication]
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Co-Autor*innen der Med Uni Graz
El-Shabrawi-Caelen Laila
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Abstract:
Intralymphatic histiocytosis is a rare condition characterized by the presence of dilated lymphatic vessels containing aggregates of mononuclear histiocytes (macrophages) within their lumina. The phenomenon seems to occur almost exclusively within the reticular dermis. Although its pathogenesis remains uncertain, there has been speculation about the possible relationship between intralymphatic histiocytosis and intravascular reactive angioendotheliomatosis. In addition, several examples historically have been associated with rheumatoid arthritis. We describe our experience with 16 cases of intralymphatic histiocytosis. Clinically, the lesions were located predominantly on the upper and lower limbs, and they consisted of asymptomatic and poorly demarcated erythematous plaques and livedo reticularis-like lesions. They were characterized histopathologically by dilated vascular structures involving the reticular dermis. Some of these dilated vessels had empty lumina, whereas others contained variable number of mononuclear histiocytes. An inflammatory response of variable intensity from case to case was also present in the adjacent dermis. The dilated vessels exhibited thin walls with irregular shapes, and a single discontinuous layer of flat endothelial cells lined their lumina. Immunohistochemically, the endothelial cells lining the dilated lumina expressed immunoreactivity for CD31, CD34, podoplanin, D2-40, Lyve-1, and Prox-1, which confirmed their nature as lymphatic endothelial cells. Intralymphatic mononuclear histiocytes expressed CD68 (PGM1), although some cases also had variable immunoexpression for myeloperoxidase, CD31, and podoplanin. In the 4 cases that employed double immunohistochemistry, with podoplanin + CD68 (PGM1) or with Lyve-1 + CD68 (PGM1), each marker highlighted their specific target cells unequivocally; the endothelial cells expressed podoplanin or Lyve-1 immunoreactivity, and intralymphatic histiocytes showed CD68 (PGM1) immunoexpression. Our findings expand on the previously described morphologic and immunohistochemical features of intravascular histiocytosis. We also discuss the possible relationship between intralymphatic histiocytosis and the so-called reactive intravascular angioendotheliomatosis.
Find related publications in this database (using NLM MeSH Indexing)
Aged -
Aged, 80 and over -
Dermis - blood supply Dermis - pathology
Eyelid Diseases - pathology
Female -
Hemangioendothelioma - pathology
Histiocytes - metabolism Histiocytes - pathology
Histiocytosis - metabolism Histiocytosis - pathology
Humans -
Lymphatic Vessels - pathology
Male -
Membrane Glycoproteins - metabolism
Middle Aged -
Phosphoglucomutase - metabolism
Vascular Neoplasms - pathology

Find related publications in this database (Keywords)
intralymphatic histiocytosis
intralymphatic macrophages
reactive intravascular angioendotheliomatosis
intravascular lymphoma
lymphatic endothelial cell markers
immunohistochemistry
rheumatoid arthritis
breast carcinoma
joint prosthesis inflammatory reaction
CD31
CD34
D2-40
podoplanin
Lyve-1
Prox-1
CD68
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