Elsevier

Human Pathology

Volume 33, Issue 10, October 2002, Pages 1023-1029
Human Pathology

Original Contributions
Angiolymphoid hyperplasia with eosinophilia: Evidence for a T-cell lymphoproliferative origin*

https://doi.org/10.1053/hupa.2002.128247Get rights and content

Abstract

Angiolymphoid hyperplasia with eosinophilia (ALHE) is commonly regarded an angioproliferative process characterized by the presence of prominent, bizarrely shaped blood vessels. These vessels are accompanied by an inflammatory infiltrate that is thought to be a reactive component. Both the cell of origin and the pathogenesis of ALHE remain controversial. To define the histogenesis of this disorder, we analyzed the phenotypic and genotypic profile of the inflammatory infiltrate in ALHE by immunohistochemistry and T-cell receptor gene rearrangement by polymerase chain reaction (PCR) and denaturing gradient gel electrophoresis, as well as automated high-resolution PCR fragment analysis. Five of 7 ALHE patients displayed a clonal T-cell population and proliferative T-cell activity in lesional tissue. Most of these cases followed a protracted and therapy-reluctant course with recurrences. These data suggest that ALHE or a subset of ALHE cases harboring a clonal T-cell population may represent a T-cell lymphoproliferative disorder of a benign or low-grade malignant nature. HUM PATHOL 33:1023-1029. Copyright 2002, Elsevier Science (USA). All rights reserved.

Section snippets

Patients and tissues

Seven formalin-fixed and paraffin-embedded skin biopsies of 7 ALHE patients from the archives of 4 institutions (Departments of Dermatology of University Hospital Zurich, Centre Hospitalier Universitaire Vaudois, and University of Munich and the Department of Pathology, Cantonal Hospital Münsterlingen) were analyzed. The clinical data are given in Table 1. The male-to-female ratio was 6:1, and the median age was 38 years (range, 19 to 66). Eosinophilia of peripheral blood was detected in 2 of

Immunohistochemistry

Analysis of the antigenic phenotypes within the lymphocytic compartment revealed that most (>80%) of the lymphocytic cells (representing about 50% of all inflammatory cells) expressed a CD3+, CD4+, CD43+, and CD45RO+ phenotype, corresponding to the characteristic surface profile of activated T-helper cells (Fig 4).

. Reactivity of about 50% of inflammatory cells for the pan-T-cell marker CD3. (APAAP; original magnification ×200.)

A minority of T cells (10% to 20%) belonged to the CD8+ cytotoxic

Discussion

Using 2 highly specific and sensitive molecular biological methods—PCR-DGGE and AHRFA—clonal populations of T cells were found in 5 of 7 cases of ALHE. PCR-based methods have proven to be reliable and useful tools in the diagnosis of cutaneous T-cell lymphomas (CTCLs), such as mycosis fungoides and Sezary's syndrome, recognizing clonal lymphocytic populations with a sensitivity >90%. Because all neoplasms of nonlymphocytic origin and the vast majority of benign inflammatory diseases are

Acknowledgements

Note Added in Proof. Recently, Chim et al reported the presence of a clonal T-cell population in a case of Kimura's disease (Am J Surg Pathol 2002;26:1083-1086).

References (32)

  • MV Botet et al.

    Angiolymphoid hyperplasia with eosinophilia: Report of a case and a review of the literature

    J Dermatol Surg Oncol

    (1978)
  • PG Henry et al.

    Angiolymphoid hyperplasia with eosinophilia

    Arch Dermatol

    (1978)
  • L Requena et al.

    Cutaneous vascular proliferation, Part II. Hyperplasias and benign neoplasms

    J Am Acad Dermatol

    (1997)
  • T Kimura et al.

    Unusual granulation combined with hyperplastic changes of lymphatic tissue

    Trans Soc Pathol Jpn

    (1948)
  • PB Googe et al.

    Kimura's disease and angiolymphoid hyperplasia with eosinophilia: Two distinct histopathologic entities

    J Cutan Pathol

    (1987)
  • SD Helander et al.

    Kimura's disease and angiolymphoid hyperplasia with eosinophilia: New observations from immunohistochemical studies of lymphocyte markers, endothelial antigens, and granulocyte proteins

    J Cut Pathol

    (1995)
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    *

    Address correspondence and reprint requests to Werner Kempf, MD, Department of Dermatology, University Hospital Zurich, Zurich, CH-8091, Switzerland.

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