Pathology
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Atlas of dermatopathology
Josef Feit, Hana Jedličková, Günter Burg, Luděk Matyska, Spasoje Radovanovic et al.
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+ Introduction
+ Literature
+ Support
+ Skin biopsy
+ Non-tumorous skin diseases
+ Skin tumors
+ Cysts of the skin and subcutis
+ Soft tissue lesions
+ Some lesions of the oral cavity
+ Dermatovenerology
+ Collection of histological slides by prof. Günter Burg
+ Collection of histological slides by prof. Werner Kempf
+ Collection of histological slides of dr. Ram Chandra Adhikari
T cell receptor gamma/delta chain positive cutaneous lymphoma
Introduction

Most peripheral T cell lymphomas express the clone specific alpha/beta heterodimer. T lymphocytes bearing the gamma/delta heterodimer usually consist of less than 5% of the peripheral blood lymphocytes and show an increase in distinct infectious conditions like leprosy. About 3% of CDTL arise form the gamma/delta positive clone of lymphocytes, showing primary or preferential homing into the skin.

Clinical signs
  • simulate MF or pagetoid reticulosis (cases of so called generalized pagetoid reticulosis of Ketron Goodman belong to this category)
  • clinical course is highly progressive in most cases, but may also be slowly progressive over many years
  • subcutaneous, pagetoid, erythrodermic and tumorous forms exist
  • subcutaneuous form resembles panniculitis, but the clinical course is more aggresive than that of subcutaneous panniculitis-like lymphoma of alpha/beta cell type
Histology

The infiltrate is localized within the upper dermis or epidermis (sometimes epidermotropic: pagetoid type) or in the subcutaneous tissue (panniculitic type); consists of the medium-sized to large pleomorphic cells with abundant cytoplasm. Tissue necroses and vascular invasion are common.

Immunophenotype and genetic features:

  • CD3+, CD43+, CD45RO+, CD4+/-, CD8+/-, CD56+/-, CD5-, CD30-, CD15-
  • TIA-1, granzyme B, granzyme M and perforin are usually positive
  • hallmark: positivity for the gamma/delta TCR (and lack betaF1 epitope of the alpha/beta receptor)

Subcutaneous panniculitis-like lymphoma gamma/delta (6000)