Benign atypical intravascular CD30+ T-cell proliferation: a recently described reactive lymphoproliferative process and simulator of intravascular lymphoma: report of a case associated with lichen sclerosus and review of the literature.

OBJECTIVES: Intravascular accumulations of atypical large lymphoid cells are a rare finding in skin biopsy specimens and raise the suspicion for intravascular lymphoma. The intravascular accumulation of atypical large CD30+ T cells, however, as a reactive process is very uncommon in the skin, with only four cases documented so far in the literature. This condition, referred to as benign intravascular atypical CD30+ T-cell proliferation, has been associated with chronic inflammation after trauma.

METHODS: We report on a case of atypical intravascular CD30+ T-cell proliferation in a patient with ulcerated lichen sclerosus on the foreskin, discuss the differential diagnoses, propose diagnostic criteria, and review the literature on this uncommon reactive intralymphatic CD30+ T-cell lymphoproliferation.

RESULTS: The atypical intravascular CD30+ T-cell proliferation is characterized by the accumulation of large CD30+ polyclonal T cells within lymphatics in close vicinity to ulceration or an inflammatory skin disease. There is no association with Epstein-Barr virus infection.

CONCLUSIONS: This benign cutaneous lymphoproliferation needs to be distinguished from intravascular T-cell lymphoma, particularly from the intravascular variant of anaplastic large cell lymphoma. Obstruction of lymphatics due to lichen sclerosus with disrupted immune cell trafficking may result in the accumulation of activated CD30+ lymphocytes.