title: Angioinvasive lymphomatoid papulosis: a new variant simulating aggressive lymphomas.
abstract: Lymphomatoid papulosis (LyP) belongs to the spectrum of primary cutaneous CD30-positive lymphoproliferative disorders. Clinically, LyP is characterized by a variable number of self-healing papulo-nodular lesions, with the typical waxing and waning course. Histologically, 4 types (A, B, C, and D) have been delineated. Angioinvasive growth and large ulcers are rare findings in LyP and simulate aggressive lymphoma. We retrospectively analyzed the clinicopathologic and molecular features of angioinvasive LyP in a series of 16 patients. This new form of LyP is characterized by oligolesional papules that rapidly ulcerate and evolve into large necrotic eschar-like lesions with a diameter of 1 to 4 cm and an angiocentric and angiodestructive infiltrate of small-sized to medium-sized atypical lymphocytes expressing CD30 and frequently CD8. As in other forms of LyP, the lesions underwent spontaneous regression after a few weeks. Recurrences were common, but the prognosis was excellent with no extracutaneous spread or disease-related deaths. Complete remission occurred in 9 of 16 patients (56%). This LyP variant should be distinguished from aggressive forms of angiocentric and angiodestructive and cytotoxic T-cell lymphomas. We propose the term LyP type E for this clinically and histologically unusual variant.
authors.affiliation: Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland. werner.kempf@access.uzh.ch
authors.forename: Werner
authors.initials: W
authors.lastname: Kempf
authors.forename: Dmitry V
authors.initials: DV
authors.lastname: Kazakov
authors.forename: Leo
authors.initials: L
authors.lastname: Schärer
authors.forename: Arno
authors.initials: A
authors.lastname: Rütten
authors.forename: Thomas
authors.initials: T
authors.lastname: Mentzel
authors.forename: Bruno E
authors.initials: BE
authors.lastname: Paredes
authors.forename: Gabriele
authors.initials: G
authors.lastname: Palmedo
authors.forename: Renato G
authors.initials: RG
authors.lastname: Panizzon
authors.forename: Heinz
authors.initials: H
authors.lastname: Kutzner
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D018414:CD8-Positive T-Lymphocytes / Q000378:metabolism / Q000473:pathology; D002648:Child; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D017730:Ki-1 Antigen / Q000378:metabolism; D017731:Lymphomatoid Papulosis / Q000235:genetics / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009365:Neoplasm Regression, Spontaneous; D012074:Remission Induction; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*
chemical_list: D017730:Ki-1 Antigen