Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Reviews and Meta-analysis
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
View Point
What’s new in Dermatology

Translate this page into:

Short Communication
PMID: 17656944

Sezary syndrome - Without erythroderma

K Abdul Samad, K Prasanna Moorthy, P Akhar Ali
 Department of Dermatology and Venereology Medical College, Trivandrum, Kerala, India

Correspondence Address:
K Abdul Samad
Department of Dermatology and Venereology Medical College, Trivandrum, Kerala
How to cite this article:
Abdul Samad K, Prasanna Moorthy K, Akhar Ali P. Sezary syndrome - Without erythroderma. Indian J Dermatol Venereol Leprol 2002;68:225-226
Copyright: (C)2002 Indian Journal of Dermatology, Venereology, and Leprology


Sezary syndrome (SS), is described as the classical triad of pruritic erythroderma, lymphadenopathy, and presence of more than 10% of circulating Sezary cells in the peripheral blood. We report on unusual case of advanced cutaneous T - cell lymphoma with classical haematological and histopathological features of Sezary syndrome, but lacking the clinical features of erythroderma.
A 66 year old man presented with asymptomatic multiple papules, plaques and nodules and with generalized lymphadenopathy. Peripheral smear showed more than 60% of Sezary cells. Skin and lymph node biopsy showed typical features of T-cell lymphoma and immunohistochemistry and CD marker studies showed the cells to be atypical T-lymphocytes. This unusual case is highlighted to denote that erythroderma need not be taken as a hard and fast criterion for diagnosing Sezary syndrome.
Keywords: Sezary Syndrome, Erythroderma, Immunohistochemistry


Sezary syndrome (SS), first described as a severe variant of cutaneous T cell lymphoma (CTCL) by Besnier and Hallopeau (1892)[1] was later defined by Sezary and Bouvrain (1938)[2] as the classical triad of pruritic erythroderma, lymphadenopathy and abnormal hyper convoluted monstrous cells in the peripheral blood. Though erythroderma is considered an invariable component of the syndrome, many recent reports showed that no single clinical feature is pathognomonic of SS and the clinical features should be correlated with histological, immunological and gene rearrangement studies.

We report case of advanced CTCL with classical haematological and histological features of SS but clinically lacking the classical component of erythrodema.

Case Report

A 66 year old man presented with multiple asymptomatic erythematous and skin coloured papules, plaques and nodules over the scalp, face, trunk and inguinal region [Figure - 1].

The lesions were hard, keratotic and fixed to the deeper structures. The lesions were of one month duration. He gave a history of jaundice for the last 6 months. He was a chronic smoker and alcoholic. Systemic examination showed generalized lymphadenopathy and a firm non- tender hepatomegaly.

The routine blood examination was normal, but the serum liver enzymes were raised. Serological investigations and serum electrophoresis were normal. Peripheral smear showed more than 60% of atypical cells with immature chromatin and inconspicuous nucleoli. FNAC from the lymph node and bone marrow aspiration cytology showed similar cells. In bone marrow trephine biopsy, there were focal infiltrations of atypical lymphocytes.

Skin biopsy from nodular lesion showed sheets of pleomorphic cells with scanty cytoplasm and hyperchroamatic nuclei and inconspicuous epidermotropism. The picture was consistent with advanced cutaneous T cell lymphoma. In immunohistochemistry the lymphocytes were CD - 45 Ro positive indicating that they were T helper cells. Thus the patient had a TNM score of T3 N3 Bt M1.


Erythroderma in Sezary syndrome may start denovo or it may follow or complicate a plaque stage of mycosis fungoides. Histological peculiarities of Sezary syndrome as distinct from the plaque/tumour stage of MF are a highly pleomorphic inflirtate, pert vascular inflirtrate, less pronounced epidermotropism and variable infiltrate density. The diverse clinical and histopathological features in advanced CTCL makes a precise differentiation between tumour stage of MF and SS difficult.[3] Based on this Trotter et al[4] have suggested that the definition of SS can be further refined by including only patients with a circulating peripheral blood population of clonal T - cells.

In our case many peculiar features created dilemma in precise classification of the condition. A sudden onset of cutaneous lesions with rapid progression was clinically suggestive of tumour de emblee ME Enormous atypical cells with hyperconvoluted nuclei in peripheral blood provide sufficient evidence for diagnosing this as Sezary syndome. Also histopathology of the cutaneous lesions and the affected lymph nodes showing highly pleomorphic inflirtrate with deeper extension and less pronounced epidermotropism are relative criteria for SS. Immunohistochemistry has proved the cells to be CD 45 + T - helper cells. Pathological features in skin, lymph node and peripheral blood with minimal invasion of bone marrow, suggested that the origin of the lymphoma was in peripheral T - lymphocytes.

Though our patient did not satisfy all the clinical criteria for the nosological entity of Sezary syndrome especially erythroderma, the skin, lymph node and peripheral blood showed the classical features of the syndrome. Hence at least in a few cases with such features, absence of erythroderma may not be taken as a hard and fast criterion to rule out the diagnosis of Sezary syndrome.

Besnier E, Hallopeau H. On the erythrodermia of mycosis fungoides. J Cutan Genito Urin Dis 1892;10:453.
[Google Scholar]
Sezary A, Bouvrain Y, Erythrodermie avec presence de cellules monstreuses dons derma et sang circulant. Bull Soc Fr Dermatol Syph 1938;45:254-60.
[Google Scholar]
Kohler S, Kim YH, Smaller BR. Histologic criteria for the diagnosis of mycosis fungoides and Sezary syndrome. J Cut Pathol 1997;24:292-297.
[Google Scholar]
Trotler MJ, Whittaker SJ, Orchard GE, et al. Cutaneous histopothology of Sezory syndrome. J Cutan Pathol 1997;24:286-291.
[Google Scholar]
Show Sections