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Logo of mjafiGuide for AuthorsAbout this journalExplore this journalMedical Journal, Armed Forces India
 
Med J Armed Forces India. 1998 October; 54(4): 376.
Published online 2017 June 26. doi:  10.1016/S0377-1237(17)30622-6
PMCID: PMC5531705

SEZARY SYNDROME-CLINICO HISTOLOGICAL CORRELATION

Dear Editor,

This refers to the report on two cases of Sezary Syndrome (MJAFI. 1998;54: 76-78).

Sezary Syndrome (SS) comprises a triad of fiery red erythroderma, generalized lymphadenopathy and circulating atypical cells with cerebriform nuclei (Sezaty cells) exceeding an absolute value of 1000/cumm or exceeding 10 percent of circulating cells [1].

Erythroderma, defined as an inflammatory skin disease which affects more than 90 percent of the body surface [2], is a characteristic feature of this syndrome, though ’multiple erythrodermic patches’ with ’butterfly rash on face’ in the first case and ’multiple erythematous scaly lesions’ in the second case of the report do not indicate the extent of cutaneous involvement, these descriptions could well suggest the clinical impression of disseminated lupus erythematosus and psoriasis respectively; more so in the absence of generalised lymphadenopathy as was in both these cases. Cytomorphologically, Sezary cell is not pathognomonic of SS, as it can be seen in many benign dermatoses like chronic eczema, psoriasis, lupus erythematosus, parapsoriasis, atopic dermatitis, vasculitis, etc. [3].

SS, sometimes described as a leukaemic variant of Mycosis Fungoides (MF), is not only clinically and hematologically distinct from it, but also distinct from a histological view, thus discouraging the inter changeable use of both these terms. The infilterate in SS is less pleomorphic than in MF; and eosinophils, plasma cells, macrophages and accessory cells are less frequent or lacking. [4]. Proliferation Kinetics of the dermal infilterate using 3H-thymidine labelling (3H index) have revealed widely distinct patterns in both [5]. Lymph nodes in MF have a higher number of interdigitating reticulum cells and the ratio of mononuclear cells having cerebriform nuclei to the interdigitating reticulum cells varies from 1:1 to 2:1, in contrast to a ratio of 5:1 to 10:1 in SS[6].

The case report serves to highlight the diagnostic dilemma a clinician might have to face when confronted with cutaneous markers of internal disease and also underscores the importance of multi disciplinary approach which is inevitable to overcome such situations.

REFERENCES

1. Arnold HL, Odom RB, james WD. Diseases of the skin (Sezary Syndrome). 8th ed. Philadelphia : WB Saunders Company. 1990:863–865.
2. Burton JL. Eczema, lichenification, prurigo and erythroderma. In: Champion RH, Burton JL, Ebling FJG, editors. Text Book of Dermatology. 5th ed. Blackwell Scientific Publications; Oxford: 1992. pp. 584–588.
3. Wieselthier JS, Koh HK. Sezary syndrome : Diagnosis, Prognosis and critical review of treatment options. J Am Acad Dermatol. 1990;22:381–401. [PubMed]
4. Imai S, Burg G, Braun-Falco O. Mycosis Fungoides and Sezary Syndrome show distinct histomorphological features. Dermatologica. 1986;173:131–135. [PubMed]
5. Sterry W, Pullmann H, Steiglder GK. Proliferation kinetics of the dermal infiltrate in cutaneous malignant lymphoma. Arch Dermatol Res. 1981;270:285–290. [PubMed]
6. Scheffer E, Meijer CJLM, Van Vloten WA, Willemzes R. A histological study of lymph modes from patients with the Sezary syndrome. Cancer. 1986;57:2375–2380. [PubMed]

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