Intralymphatic histiocytosis comprises M2 macrophages in superficial dermal lymphatics with or without smooth muscles

Noriki Fujimoto, Gen Nakanishi, Toshiaki Manabe, Taku Fujimura, Toshihiro Tanaka

Research output: Contribution to journalArticlepeer-review

4 Citations (Scopus)


Intralymphatic histiocytosis represents a rare reactive disorder, which is characterized by the accumulation of macrophages within lymphatic vessels and observed predominantly in upper extremities. The infiltration and preferential M2 differentiation of macrophage are observed in chronic lymphedema, and lymphedema is considered a causative factor of intralymphatic histiocytosis. However, what causes accumulation of histiocytes in the lymphatic vessels remains unclear, and investigation regarding the characteristics of the macrophages has not been evaluated. We present a case of intralymphatic histiocytosis, in which immunohistochemical staining for both macrophages and lymphatic vessels was performed to evaluate the nature of macrophages within lymphatic vessels and to determine the causative factor. Aggregated macrophages were shown to be M2 macrophages positive for CD68, CD163 and CD206 but negative for inducible nitric oxide synthase. Thick lymphatic vessels positive for D2-40 and α-SMA in the superficial dermis were observed. We speculate that chronic lymphedema leads to hypertrophy of lymphatic vessels with smooth muscle in the superficial dermis, which may be a kind of malformation, and these lymphatic vessels produce some chemokines that induce intralymphatic aggregation of macrophages.

Original languageEnglish
Pages (from-to)898-902
Number of pages5
JournalJournal of cutaneous pathology
Issue number10
Publication statusPublished - 2016 Oct 1
Externally publishedYes


  • CD206
  • M2
  • intralymphatic histiocytosis
  • smooth muscle
  • α-SMA

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology


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