|Year : 1991 | Volume
| Issue : 1 | Page : 29-31
Poikilodermatous mycosis fungoides and sezary synddrome
RR Mittal, A Chopra, R Popli, V Gupta
R R Mittal
Source of Support: None, Conflict of Interest: None
Four cases of poikildermatous mycosis fungoides are reported. All four had intense pruritus, progressive reticulate pigmentation, interspersed with atrophic, wrinkled, hypopigmented macules and were confirmed histopathologically. Abnormal lymphocytes were seen in case 1 and 3 in the peripheral blood film (PBF). Case I had erythroderma, lymphadenopathy and 10 percent abnormal lymphocytes in PBF and so was labeled as case of sezary syndrome.
Keywords: Poikiloderma, Mycosis Fungoides, Sezary syndrome
|How to cite this article:|
Mittal R R, Chopra A, Popli R, Gupta V. Poikilodermatous mycosis fungoides and sezary synddrome. Indian J Dermatol Venereol Leprol 1991;57:29-31
|How to cite this URL:|
Mittal R R, Chopra A, Popli R, Gupta V. Poikilodermatous mycosis fungoides and sezary synddrome. Indian J Dermatol Venereol Leprol [serial online] 1991 [cited 2020 Jun 6];57:29-31. Available from: http://www.ijdvl.com/text.asp?1991/57/1/29/3614
The Sezary syndrome was first described by Sezary and Bouvrian in 1938. It is characterised by generalised erythroderma with intense itching, peripheral lymphadenopathy and the presence of Sezary cells in the cellular infiltrate in skin and peripheral blood. Sezary cells are abnormal T helper lymphocytes ,, and have abnormal DNA content. The Sezary syndrome is now generally regarded as an erythrodermic leukaemic variant of mycosis fungoides and erythroderma is presumed to be due to vasoactive lymphokines released by Sezary Ce lls. , Sezary cells either originate in skin , or lymph nodes , and appear in blood as an overspill phenomenon. Leucopharesis or combination of steroid and chlorambucil, have been found useful in Sezary syndrome. Poikilodermatous variant of mycosis fungoides is well known.
| Case Reports|| |
A 55 year old female was suffering from repeated episodes of morbilliform eruptions since 4 years. Initially, photosensitive rash was present on the arms, face and neck. Erythroderma developed within 2 years. After repeated episodes of similar rash, there were intense itching, generalised erythema, thickening of skin, reticulate pigmentation, intermingled with atrophic, wrinkled, scaly, hypopigmented macules. These changes were more marked on the breasts, buttocks, axillae, groins and neck. Axillary lymph nodes were bilaterally enlarged. Haemoglobin was 10.6 gm % and total leucocyte count, 7600/ cmm, with differential P 55 %, L35 %, E 7 % and M 3 %. Blood urea, stools and fasting blood sugar were normal. Urinalysis revealedl0-15 pus cells per HPF, ESR was 3 mm. Peripheral blood film showed monocytic, monochromic red blood cells with adequate platelets. 10 % abnormal lymphocytes were seen. Histopathology showed degeneration of basal cells. The upper dermis was infiltrated by lymphocytes, few histiocytes and melanin filled macrophages. Few abnormal lymphocytes with hyperchromatic nuclei were seen in the dermal infiltrate and in the epidermis.
Case 2 : A 60 year old female suffering from progressively increasing reticulate pigmentation with intermingling of scaly, atrophic hypopigmented macules for the last 3 years [Figure - 1]. There were associated moderate itching, progressive weakness and loss of weight. General physical and systemic examination including routine investigations were normal. Histopathology showed atrophy of epidermis and effacement of rete-ridges with hydropic degeneration of basal cells. Upper dermal infiltrate consisted of lymphocytes, few histiocytes and melanin filled macrophages. Few lymphocytes had nuclear hyperchromasia.
Case 3 : A 53 year old female was suffering from persistent, pruritic, erythematous, papular and well defined plaques on the face for the last 4 years. Photosensitivity was present and aggravation of rash during summer was seen. 1 year back she developed progressive reticulate pigmentation interspersed with atrophic, white macules on the neck and breasts. Seborrhoea capitis was present. General physical and systemic examination including routine investigations were normal. Peripheral blood film showed 8% abnormal lymphocytes histopathology revealed atrophy of epidermis and hydropic degeneration of basal cells. Upper dermis was infiltrated by lymphocytes, histiocytes and melanin filled macrophages. Some lymphocytes showed nuclear atypia.
Case 4 : A 54-year-old female has been suffering from progressively increasing reticulate pigmentation associated with intermingled wrinkled, atrophic, hypopigmented macules on the front of chest, over the breasts, trunk, upper and lower limbs for the last 18 months. Intense itching and progressive weakness were present. General physical and systemic examinations were normal. Various readings of the ESR have been ranging from 35 to 45 mm at the first hour (Westergren). Histopathology revealed atrophy of the epidermis with effacement of rete-ridges. Hydropic degeneration of basal cells with lichenoid infiltrate was seen. Some of the cells in the infiltrate showed nuclear hyperchromasia and few such cells were invading the epidermis [Figure - 2] She had suffered from borderline tuberculoid leprosy 6 years back and was cured with 3-1/2 years treatment.
| Comments|| |
Erythroderma, reticulate pigmentation with intermingling of atrophic, hypopigmented macules and presence of abnormal lymphocytes in the dermis and epidermis led to the diagnosis of poikilodermatous mycosis fungoides in all the four cases. Presence of 10% abnormal lymphocytes, erythroderma and axillary lymphadenopathy in the first case led to the diagnosis of Sezary syndrome which represents the erythrodermic leukaemic variant of mycosis fungoides. Such cases of poikilodermatous mycosis fungoides and Sezary syndrome have not been reported earlier in the Indian literature, as far as known to us.
| References|| |
|1.||Sezary A, Bouvrian Y : Erythodermic avec presence de cellules monstreuses clans derma et sang circulant, Bull Soc Fr Dermatol Syphil, 1938; 45 254-260. |
|2.||Tasewell HF, Winkelmann RK : Sezary syndrome - a malignant reticulemic erythroderma, J Amer Med Assoc, 1961; 177 : 465-172, |
|3.||Lutzner MA, Jordan HW : The ultrastructure of an abnormal cell in sezary syndrome, Blood, 1968; 31 :719-726. |
|4.||Crossen PE, Mellor JEL, Anley AG et al. : The Sezary Syndrome : Cytogenetic studies and identification of the Sezary Cells as an abnormal lymphocyte, Amer J Med, 1971; 50: 24-34. |
|5.||Van Vloten WA, Van Duijin P, Schaberg A Cytodiagnostic use of Feulgen - DNA measure ments in cell imprints from the skin of the patients with mycosis fungoides, Brit J Dermatol, 1971; 91: 365-371. |
|6.||Edelson RL, Kirkpatrick CH, Shevach EM et al Preferential cutaneous infiltration by neoplastic thymus-derived lymphocytes. Morophological and functional studies. Ann Int. Med, 1974; 80: 685692. |
|7.||Main RA, Goodall GB, Swanson WC : Sezary Syndrome, Brit J Dermatol, 1959; 71 : 335-343. |
|8.||Miller RA, Coleman CN, Fawcett HD et al : Sezary Syndrome : A model for migration of T Lymphocytes to skin, New Eng J Med, 1980; 303 : 89-92. |
|9.||Edelson R, Facktor M, Andrews A et al : Successful management of the Sezary Syndrome, mobilization and removal of extravascular neoplastic T cells by leukapheresis, New J Med, 1974; 291 293-294. |
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|11.||Rona M MacKlE : Mycosis Fungoides in Text Book of Dermatology, 4th ed, Editors Rook A, Wilkinson DS, Ebling FJG, Champion, RH Oxford University Press, 1987; p. 1736-1749. |
[Figure - 1], [Figure - 2]