Mycosis Fungoides

MYCOSIS FUNGOIDES (syn.: granuloma fungoides, granuloma sarcomatodes, fibroma fungoides), fungoid mycosis, fungoid granuloma, a chronic general disease of inflammatory granuloma character with predominant localization on the skin in the form of highly polymorphic eruptions - eczematous foci, infiltrates, nodules, etc., - accompanied by intense itching; first described by Alibert (1812) under the name "pian fongoide". Alibert considered M. f. a disease closely related to syphilis, and only his student Bazin (1851 and later), having thoroughly developed the question of M. f., recognized it as an independent disease. The exact etiology and pathogenesis are unknown; there are four main hypotheses: 1) M.f. is a manifestation of cutaneous lymphadenia and belongs to leukemic diseases (the old view of the French school; Ranvier, Darier, Pelegatti); 2) M. f. is a lymphosarcoma (Radaeli, Fraser); 3) M. f. belongs to the so-called sarcoid granulomas, sarcoids (Bogrov, Finger, Kreibich); 4) M. f. is an infectious granuloma (Kobner, Paltauf, Lubarsch and many others) - The last view seems most convincing, confirmed by experimental data obtained in recent years by Briinauer and Chevrel. -M. f. is a rare disease, but occurs in almost all countries. According to statistics from the American Dermatological Association over 34 years, M. f. constitutes 0.025% of all skin patients; in the Breslau skin clinic - 0.017%. In the USSR, M. f. apparently occurs even less frequently than in Western Europe. The histological picture of most eruptions in the premycotic stage is completely uncharacteristic; usually partial parakeratosis, acanthosis, spongiosis and infiltration of cells are found in the epidermis; in the dermis - banal inflammatory phenomena. Only in cases of premycotic erythroderma are the histological changes similar to those of the tumor stage. In the proper skin, the entire papillary and part of the subpapillary layer is occupied by a sharply limited (from below) dense, predominantly lymphocytic infiltrate; vessels are dilated and surrounded by infiltrate, papillae are edematous. Interspersed epithelial processes are elongated; small accumulations of lymphocytes characteristic of M. f. are often found in the Malpighian layer. -The mycotic tumors consist of an abundant, highly polymorphic cellular infiltrate occupying the entire proper skin and subcutaneous tissue; the infiltrate pushes apart the connective tissue fibers so much that a thin mesh is formed from them. In the infiltrate, a special variety of lymphoid cells of the leukoblast type predominates significantly. -In most cases of M. f., at autopsy, infiltrates and tumors similar to those of the skin are found in internal organs. Most often affected are the lymphatic system and lungs; from other organs - pleura, kidneys, spleen, pancreas, gastrointestinal tract, brain, thyroid gland, heart, etc. Inoculation of animals with mycotic tissue repeatedly gave negative results. In 1924, Briinauer succeeded in implanting pieces of altered internal organs of M. f. patients into guinea pigs, causing changes in the liver, spleen and inner surface of the sternum, which histologically showed great similarity to changes in M. f. in humans. Chevrel (1928), by inoculating guinea pigs and rabbits with blood from M. f. patients, obtained the formation of nodes in internal organs similar to mycotic ones, and in these nodes she discovered a special cocco-bacillus. The latter finding has less significance, since earlier a number of authors had succeeded in finding various cocci and bacilli in the nodes and blood of M. f. patients. The experiments of Briinauer and Chevrel speak in favor of the infectious theory of mycosis fungoides. Clinically, three main forms of M. f. are distinguished: 1) the classical form, 2) the erythrodermic, or diffuse form, and 3) M. f. d'emblée.--1. The classical Alibert-Bazin form. Two periods are distinguished: the so-called premycotic period and the period of tumors. Bazin divided the entire course of M. f. into 3 stages: a) erythematous or eczematous, b) lichenoid, and c) the tumor stage. At present, the first two stages are combined into one - the premycotic period. The duration of the latter varies greatly: from several months to decades. Premycotic eruptions are very diverse, they always (with extremely rare exceptions) are accompanied by intense itching, which in some cases appears long before the first eruptions. Sometimes prodromal phenomena precede the eruptions: depressed general condition, loss of appetite, phenomena from the gastrointestinal tract, elevated temperature, etc. Primary eruptions are of erythematous, eczematous or urticarial character; they appear on the entire body or on limited areas. Due to merging, peripheral spread and partial regression of eruptions, a very variegated picture is often obtained in terms of both the location of the rash and its color. Subsequently, limited dense merging flat infiltrates of reddish color of various sizes gradually begin to appear on the skin, which after some time either resolve, leaving pigmentation, or turn into nodules. In some cases, infiltrates are covered with abundant scales; then the picture of the disease resembles psoriasis. Sometimes premycotic eruptions strongly resemble parapsoriasis, neurodermatitis and some other dermatoses. -There is no sharp boundary between the premycotic stage and the period of tumors, since usually both polymorphic premycotic eruptions and nodes are present simultaneously. Tumors form on both apparently healthy skin and on the surface of infiltrates. In a short time (from several days to several weeks), nodes can reach considerable size (up to a child's head). Rapid growth is characteristic of M. f. Appearing quickly, nodes can just as quickly disappear. The shape of the nodes is often similar to that of tomatoes; sometimes the tumors have the shape of a mushroom cap. Their surface is bright or bluish-red in color, smooth, shiny [see separate table (for the article Soft chancre), fig. 8]. The node either resolves without ulcerating, leaving pigmentation, or ulcerates. Sometimes nodes are arranged in groups; in these cases, large ulcerative surfaces can form. The edges of ulcers are infiltrated and partially everted. Upon healing, a delicate scar forms. In some cases, tumors grow deep into muscles and bones. 2. The erythrodermic (Hallopeau, Besnier), or diffuse (Leredde) form proceeds in the form of diffuse exfoliative erythroderma (see Erythroderma), in some cases erythroderma is primary: it is not preceded by any other premycotic eruptions. Unlike the usual highly polymorphic picture of M. f., in this form the clinical picture is very uniform. Periodically improving and worsening, erythroderma can continue for a very long time until the first infiltrates, and then tumors appear. -3. M. f. d'emblée (Vidal, Brocq) is characterized by the appearance of tumors without preceding premycotic eruptions. As a variety of this form, the so-called reverse type of M. f. is also distinguished: first tumors appear, and then after a longer or shorter time - eruptions, which are usually in the premycotic period (here they are actually postmycotic). The diagnosis of M. f., excluding the clinically characteristic tumor stage, is often difficult, especially in the initial period. Individual premycotic eruptions can simulate eczema, Vidal's ringworm, psoriasis, parapsoriasis and other skin diseases. The following help in making the correct diagnosis in such cases: 1) polymorphism of eruptions, 2) sharp boundaries and often figurate outlines of the latter, 3) very intense itching, which sometimes also affects clinically healthy skin; the diagnosis is confirmed by the appearance of dense infiltrates. In the period of tumors, especially in M. f. d'emblée, the differential diagnosis with gummatous syphilis can be difficult; the characteristic appearance of the nodes ("tomatoes"), their multiplicity, as well as the usually simultaneously present polymorphic premycotic eruptions help in the diagnosis. The histological picture of infiltrates and tumors is also of diagnostic importance. The blood picture (monocytosis, eosinophilia) has little diagnostic significance. The prognosis for complete recovery is always unfavorable: M. f. always ends in death, although patients often live for decades with good general condition. The prognosis is worse for the erythrodermic form and for M. f. d'emblée, since with them a fatal outcome often occurs rapidly. -Treatment. The best results are obtained by applying X-rays to the affected areas of the skin (u4-u3H.E.D. with a 1-2 mm aluminum filter; 2-3 sessions with 7-14 day intervals). X-ray therapy is indicated both in the nodal stage and in the premycotic stage. M. f. is a disease very sensitive to X-rays. Long-term use of arsenic is also recommended. Any local therapy has only symptomatic significance.