Pathology
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Atlas of dermatopathology
Josef Feit, Hana Jedličková, Günter Burg, Luděk Matyska, Spasoje Radovanovic et al.
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Contents
Authors
ExpSys: non-tumors
ExpSys: tumors
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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
Sézary syndrome
Introduction

Leukemic, erythrodermic, low grade malignant, peripheral T cell lymphoma involving peripheral lymph nodes in most cases.

Clinical signs
  • generalised disease
  • multiple infiltrated lesions or pruritic erythroderma
  • peeling, hyperpigmentation
  • facies leonina
  • hyperkeratoses of the palms, soles, alopecia, onychodystrophy
  • pruritus
  • lymphadenopathy, leukocytosis with Sézary cells, infiltration of the bone marrow, hepatosplenomegaly

Sézary syndrome (923)

Pictures

Sézary syndrome: facies leonina Sézary syndrome, Clinical picture (923)

Histology

Superficial epidermotropic lymphocytic infiltrate with scattered large, atypical lymphocytes with hypercovoluted nuclei; little or no spongiosis, usually some acanthosis. Eosinophils are sometimes present, but their number is usually less than in mycosis fungoides. The infiltrate is usually less pleomorphic than in mycosis fungoides.

Immunophenotype and genetic features:

  • CD2+, CD3+, CD5+,CD45RO+, CD8-, CD30-
  • clonality is present in most cases in skin infiltrates, peripheral blood and involved lymph nodes

Sézary syndrome (12738)

Sézary syndrome (12736)

Pictures

Sézary syndrome: Sézary syndrome, HE 40x (2506) Sézary syndrome, HE 100x (2505)

Another case: Sezary syndrome with Pautrier's microabscesses, HE 60x (12736)

Another case: Sezary syndrome with Pautrier's microabscesses, HE 60x (12737)

Another case: Sezary syndrome with Pautrier's microabscesses and epidermotropism, HE 60x (12738)

Another case: Sezary syndrome, HE 20x (4607) Sezary syndrome, CD3 20x (4606)

Another case: Sezary syndrome, HE 60x (14262)

Another case: Sézary syndrome, HE 10x (1687) Sézary syndrome, HE 20x (1688) Sézary syndrome, HE 40x (1689)

Pre-Sézary syndrome: Pre-Sézary syndrome, HE 40x (2187)