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Angioinvasive Lymphomatoid Papulosis: A New Variant Simulating Aggressive Lymphomas

Kempf, Werner MD*,†; Kazakov, Dmitry V. MD, PhD; Schärer, Leo MD§; Rütten, Arno MD§; Mentzel, Thomas MD§; Paredes, Bruno E. MD§; Palmedo, Gabriele PhD§; Panizzon, Renato G. MD; Kutzner, Heinz MD§

American Journal of Surgical Pathology: January 2013 - Volume 37 - Issue 1 - p 1–13
doi: 10.1097/PAS.0b013e3182648596
Original Articles

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.

*Kempf und Pfaltz, Histologische Diagnostik

Department of Dermatology, University Hospital, Zürich

Dermatology, Centre Hospitalier Universitaire Vaud, Lausanne, Switzerland

Department of Pathology, Faculty of Medicine in Pilsen, Charles University in Prague, Czech Republic

§Dermatopathologie Friedrichshafen, Germany

Case 1 was presented by 1 of the authors (W.K.) at the Self Assessment Course of the XXIII annual meeting of the International Society of Dermatopathology (ISDP), 2002, in Stresa, Italy.

Conflicts of Interest and Source of Funding: The authors have disclosed that they have no significant relationships with, or financial interest in, any commercial companies pertaining to this article.

Correspondence: Werner Kempf, MD, Kempf und Pfaltz Histologische Diagnostik, Seminarstrasse 1, CH-8042 Zürich, Switzerland (e-mail: werner.kempf@access.uzh.ch).

© 2013 Lippincott Williams & Wilkins, Inc.