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Angioinvasive Lymphomatoid Papulosis: A new variant simulating aggressive lymphomas


Kempf, Werner; Kazakov, Dmitry V; Schärer, Leo; Rütten, Arno; Mentzel, Thomas; Paredes, Bruno E; Palmedo, Gabriele; Panizzon, Renato G; Kutzner, Heinz (2013). Angioinvasive Lymphomatoid Papulosis: A new variant simulating aggressive lymphomas. American Journal of Surgical Pathology, 37(1):1-13.

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.

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.

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Additional indexing

Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Dermatology Clinic
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2013
Deposited On:14 Dec 2012 09:58
Last Modified:05 Apr 2016 16:09
Publisher:Lippincott, Williams & Wilkins
ISSN:0147-5185
Additional Information:This is a non-final version of an article published in final form in (provide complete journal citation)Kempf, Werner; Kazakov, Dmitry V; Schärer, Leo; Rütten, Arno; Mentzel, Thomas; Paredes, Bruno E; Palmedo, Gabriele; Panizzon, Renato G; Kutzner, Heinz (2012). Angioinvasive Lymphomatoid Papulosis: A new variant simulating aggressive lymphomas. American Journal of Surgical Pathology:Epub ahead of print.
Publisher DOI:https://doi.org/10.1097/PAS.0b013e3182648596
PubMed ID:23026936

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