[Mycosis fungoides--the practionner confronted with the nosological, diagnostic and prognostic problems of epidermotrophic lymphomas (author's transl)]. / Mycosis fongoïde--le praticien devant les problèmes nosologiques, diagnostiques, pronostiques des lymphomes épidermotropes.

The authors have studied 39 cases of mycosis fungoides in the last 3 years. The confrontation between clinical and histological data remains the best means of diagnosis and prognosis. The classical classification into three clinical forms must be maintained; laboratory investigations do not yet supply practical information for prognosis and therapy. At present, the prognosis cannot be established otherwise than on clinical grounds; a pragmatic staging method is needed.

Preferential replication of Sézary cells in the epidermis.

Skin biopsies from three patients with Sézary Syndrome have been labeled in vitro with H-3Thymidine. Labeled cells have been counted in semithin Epon embedded sections and characterized by electron microscopic autoradiography. The ratio of labeled Sézary cells to total lymphocytes was two to four times higher in the epidermis than in the dermis. It is concluded that Sézary cells replicate in the skin and that epidermal cells seem to possess new blastogenic properties in Sézary Syndrome.

[Sézary's syndrome]. / Le syndrome de Sézary

In Sézary's syndrome (SS), cutaneous manifestations are associated with an abnormal mononuclear cell in the blood. Clinically, the differential diagnosis with Mycosis fungoides is impossible. However, some of the symptoms are more in favor of SS, such as leonin face, palm and sole hyperkeratosis and changes in pigmentation. The diagnosis of SS rests upon the identification on ordinary blood smears of a lymphocytic cell, the size of which is variable and which exhibits a grooved, folded and lobulated nucleus. This cell is negative for both esterases and peroxydases. It is not a monocyte. Large cells have big nuclei with tetraploid DNA values, whereas small variants have near diploid values. Chromosome analysis may reveal near hypertetraploid, hyper or hypodiploid counts. Marker chromosomes have been seen which could be common to all types. However, many cases are known in which the karyotype was normal. In addition, there are cases in which chromosome analysis was impossible due to poor responses of the Sézary cells to PHA. As regards surface markers, there is general agreement that Sézary's cells are non-B lymphocytes. In some cases, the cells had both T markers (antigen and E-rosettes), whereas in others, only one (antigen) was expressed. In still other cases, the abnormal blood cell had neither B nor T markers. In at least one case, the cells had both B and T surface receptors. The diversity of the Sézary's cell would be explained by the immunologic pressure exerted upon skin lymphocytes by the epidermis due to a change in the antigenicity of this tissue in relation to ageing or to the presence of exogenous chemicals, or to physically induced mutations. Recent studies from this laboratory, using autoradiography after incorporation of tritiated thymidine, have shown labelled lymphocytes in the skin of three out of four cases of SS. These results suggest that cutaneous manifestations in SS, as well as in Mycosis fungoides, are comparable to a chronic Graft-Versus-Host reaction (GVH).

[Peculiarities of lymphocytes during dermatosis. Ultrastructural study of 100 cases]. / Particularités des lymphocytes au cours des dermatoses. Etude ultrastructurale de 100 cas

Peripheral blood lymphocytes from one hundred patients with skin disorders have been examined under the electron microscope. The bloods of 20 donors were used as controls. One observed: Branched Tubular Structures (so-called Lupus type inclusions) in 8 cases (4 systemic Lupus Erythematosus out of 15; I mixed connective tissue disorder; 3 cutaneous lymphomas). As regards Lupus, these findings are in agreement with those already reported in the literature. However, the presence of such inclusions in Lymphomas calls attention once again on the fact that the lymphocyte appears like a possible common denominator to both autoimmune diseases and lymphomas; Intented or cerebriform nuclei in 12 cases, all lymphomas, including 8 cases of Sezary Syndrome out of 8 and 4 Mycosis Fungoïdes out of 12; Lamellar type inclusions, similar to those described by Hovig, in 13 cases (connective tissue disease 1, lymphomas 2, psoriasis 4, miscellaneous 4, healthy controls 2). Their meaning is unclear; they may be artifacts.

[Examination of the peripheral blood in 100 dermatology cases]. / Examen du sang périphérique dans 100 cas dermatologiques

Routine examination under the electron microscope of lymphocytes from peripheral blood in 100 patients with skin disease and 20 healthly controls, was carried out seeking branched tubular inclusions of lupus type and lymphocytes with an indented or cribriform nucleus, and gave the following results: -Presence of inclusions of lupus type in 8 cases (4 cases of systemic lupus out of 15 examined, 1 case of sclerodermatomyositis, 3 lymphomas of the skin). These results confirm previous studies carried out in cases of collagen disease and, in addition, demonstrate branched tubular inclusions in lymphomas, they emphasize the importance of the lymphocyte as common denominator in man, in both auto-immune diseases and in lymphoma.-The presence of cells with an indented, or cribriform nucleus in 12 cases out of 100 patients (8 cases of Sézary syndrome out of 8; 4 cases of mycosis fungoides out of 12). Lymphoma deposits were never found outside the skin. -A second type of cytoplasmiclymphocytic inclusion, with a lamellar appearance, has already been described by Hovig and was noted in 13 cases out of 120 biopsies in various diseases and in 2 healthy controls. These were perhaps artifacts.