BRITISH MEDICAL JOURNAL
409
18 AUGUST 1979
rapidly with symptomatic treatment. Physical signs were conspicuously lacking, and no child was detained overnight. During the previous fortnight, the staff recalled, there had been a few cases of a similar type. Some of these children were thought in retrospect to have had attacks of gastroenteritis, for November is the month in which this infection begins to increase in schoolchildren. The next day, 13 more children were affected, and 20 others had recurrences. The staff noticed that while the affected children were being examined other children, hitherto quite well, at once began to complain of symptoms. Meanwhile contaminated food or water, chemical toxins, and common infections were being effectively ruled out as causes. On Friday the school was closed, and no cases were reported from home on that day or at the weekend. Nevertheless, when the school reopened the succeeding Monday 16 new cases and 29 recurrences were recorded, but they proved to be the final cluster of cases. Only a handful more occurred in the rest of the week. In all 196 pupils out of 1663 were affected-18o0 of the girls and 5 o of the boys. The oldest children, aged 10-11, were most affected, and no teachers were ill. Such outbreaks, rare (or at least seldom reported) in the first half of this century, seem lately to have become more frequent. Sirois2 reviewed 20 recorded episodes up to 1973. Many of the school epidemics had occurred in Britain3-6 and in the United States,7-" but schools in Ghana,'2 Malaysia,'3 14 Uganda and Tanzania,'5 and Northern Ireland'6 have also had outbreaks. Similar events have been recorded in children's bands marching, overclad, at football games in the United States'7 18 and in a similar band of young people, including drum majorettes, near Newcastle upon Tyne'9; among nurses at a leprosarium in Japan20; and at factories in the United States2' and in Singapore.22 Many of the school outbreaks have features in common that should suggest a positive diagnosis of epidemic hysteria. Girls make up 70-1000o of those affected; the first cases occur in classes of 12-14 year olds, though younger children may later develop symptoms; the staff and parents or others at home are very seldom ill. The symptoms greatly outweigh any abnormal physical signs, the most common being abdominal pain, faintness, nausea, headache, weakness, and itching. Some girls may be seen to overbreathe. In some cultures more dramatic phenomena occur, such as weeping, wailing, screaming, giggling, running, and claims to have seen ghosts. Spread often seems to be by direct vision: children succumb from seeing other children affected. Another characteristic is that individuals recover very rapidly. The outbreak may, as Sirois says,23 either be explosive (with or without a trigger in the form of one or two mentally or physically sick girls) or slowly cumulative. Sometimes the epidemic may occur in waves, and in day schools very few or no cases occur at intervening weekends. In many outbreaks there has been some cause for underlying anxiety, such as a recent polio epidemic, an unsatisfactory headmaster, impending examinations, or a cultural conflict between home and school. For the school such an epidemic is alarming. The staff may not be aware of the possibility of epidemic hysteria or may be reluctant to entertain the possibility. The search for evidence of infection, especially by viruses, for contamination of food or water, and for toxic gases may take a long time. Meanwhile alarm among parents grows, and the presence of doctors, inspectors, firemen, or police at the school, and reports in the press, extend and prolong the outbreak. Firm reassurance based on a positive diagnosis, without too long a delay to exclude every other conceivable cause, may be very successful.
Failing that, the best policy is that recommended by Johann Weyer24 in the Rhineland in the sixteenth century for dealing with bewitched and demoniacal people in convents: "It is necessary first of all that they all be separated and that each of the girls be sent to her parents or relatives." In other words, for the time being the school should be closed. Figueroa, M, Caribbean Epidemiology Centre Surveillance Report, 1979, 5, No 4. Sirois, F, L'Union Midicale du Canada, 1973, 102, 1906. 3Miller, R, and Raven, M, British Medical_Journal, 1936, 1, 1242. 4Moss, P D, and McEvedy, C P, British Medical_Journal, 1966, 2, 1295. 5 McEvedy, C P, Griffith, A, and Hall, T, British MedicalyJournal, 1966, 2, 1300. 6 Benaim, S, Horder, J, and Anderson, J, Psychological Medicine, 1973, 3, 366. Goldberg, E L, Journal of School Health, 1973, 43, 362. 8 Levine, R J, et al, Lancet, 1974, 2, 1500. 9 Polk, L D, Clinical Pediatrics, 1974, 13, 1013. 10 Nitzkin, J L, Journal of the Florida Medical Association, 1976, 63, 357. "Helvie, C 0,JYournal of School Health, 1968, 38, 505. 12 Adomakoh, C C, Ghana 1973, 12, 407. 13 Teoh, J-I, Singapore Medical3Journal, 1975, 16, 301. 14 Tan, E S, Medical3Journal of Malaya, 1963, 18, 72. 15 Ebrahim, G J, Clinical Pediatrics, 1968, 7, 437. 16 Lyons, H A, and Potter, P E,yournal of the Irish Medical Association, 1970, 63, 377. 17 Pfeiffer, P H, Journal of the Maine Medical Association, 1964, 55, 27. 18 Levine, R J,J_ournal of the American Medical Association, 1977, 238, 2373. 19 Smith, H C T, and Eastham, E J, Lancet, 1973, 2, 956. 20 Ikeda, Y, Psychiatry, 1966, 29, 152. 21 Champion, F P, Taylor, R, Journal of the South Carolina Medical Association, 1963, 59, 351. 22 Chew, P K, Phoon, W H, and Mae-Lim, H A, Singapore Medical3Journal, 1976, 17, 10. 23 Sirois, F, Acta Psychiatrica Scandinavica, 1974, suppl p 252. 24 Quoted in Zilboorg, G (in collaboration with G W Henry), A History of Medical Psychology, p 221. New York, W W Norton, 1941. 2
Medical_Journal,
Mycosis fungoides-unsolved problems What is mycosis fungoides ? The double allusion in the nineteenth century name does not imply any link with a fungus infection. Though generally thought to be a malignant T cell lymphoma arising in the skin, its exact place among the lymphomas is obscure.' One curious feature is epidermotropism-the affinity of its lymphoma cells for the epidermis. Some have also suggested that mycosis fungoides may be a disordered immunological process, a disease of antigen persistence.2 The two concepts are not necessarily mutually completely exclusive. For some investigators mycosis fungoides includes any lymphoma when it affects the skin, for others any lymphoma which seems to arise in the skin. Nevertheless, there is more to be said for restricting the term to an entity characterised by its clinical picture and by the distribution, cytology, and ultrastructure3 of the cellular infiltrate in the skin. Unfortunately, these various criteria may not all be satisfied in every case; and no single one seems invariable. For example, the histological appearances of a very aggressive lymphoma type may be associated with an apparently good prognosis in lymphomatoid papulosis, and cells with the ultrastructural appearances of those in mycosis fungoides may be found in benign inflammatory dermatoses. Classical mycosis fungoides4 5 starts with a rather characteristic eruption of fixed erythematous plaques, which are not apparently grossly infiltrated and are devoid of the more florid histological changes found later in the disease. After months, years, or decades the more characteristic infiltrated plaques and even
410
tumours may arise, and may lead on within a year or two to death from sepsis or from systemic disease. Clinical confusion arises about the so-called premycotic eruptions, and also around the "d'emblee" forms of the disease, which do not show the early phase. The term parapsoriasis is hallowed by tradition, but unless qualified it is best avoided. Undoubtedly many patients develop fixed erythematous lesions which may not clear but never develop into mycosis fungoides. This eruption is sometimes called benign parapsoriasis or, perhaps better, persistent superficial dermatitis.4 Other cases are superficially rather similar, but closer inspection by an experienced eye shows some of the tell-tale signs of true mycosis fungoides: the varying hue of the lesions, the presence of atrophy, and telangiectasia, even amounting to poikiloderma. This type is sometimes given the name premycotic type of parapsoriasis. Nevertheless, this is perhaps a misnomer because it seems likely that the eruption has been mycosis fungoides from the beginning.4 The distinction between the two processes may be extremely difficult clinically. The presence or absence of itching is not a reliable guide. Doubt often remains even after repeated examinations and several biopsies. But this is not equivalent to saying that the diseases really overlap or that the persistent superficial dermatitis can really evolve into mycosis fungoides, though the possibility has not been excluded. It more reflects our lack of adequate diagnostic criteria. The nature of the d'emblee cases is also in doubt. These start with infiltrated plaques or tumours and run their course more rapidly. Histologically these may be indistinguishable from classical mycosis fungoides but some such patients have B-cell lymphomas rather than the T-cell proliferation of mycosis fungoides and deserve to be kept apart. Likewise patients who present with an erythroderma deserve to be separated off. Some are examples of the Sezary syndrome with its distinctive clinical picture and characteristic "cellules monstreuses" in the peripheral blood. The relationship of the Sezary syndrome to mycosis fungoides, of which it may be a leukaemic variant, is undecided.6 7 The history of classical mycosis fungoides is extremely variable, and the prognosis, at least in the early stages, is far from gloomy, though it may spoil life without destroying it. In many cases the "premycotic" eruption may never evolve into the more specific infiltrative phase, even after 50 years. In Samman's British series5 only 8% of patients had died ofthe disease within 10 years of onset, though once the infiltrated plaques and tumours have arisen the prognosis is far from good. The presence of enlarged lymph nodes, whether from specific infiltration or just from inflammatory changes, adversely affects the prognosis. In Britain deaths from the disease are usually due to secondary infection, and a distressing end state this may be; systemic disease, with the possible exception of spread to the lymph nodes, is rare. Interestingly, figures from the United States give a different emphasis.8 9 There systemic disease is much more common. This may reflect a different pattern of referral to the major centres, or be due to a true heterogeneity of the disease itself. Quite possibly more than one disease process is masquerading under the clinical picture of classical mycosis fungoides. Various criteria for staging of mycosis fungoides have been published'° 11 comparable with the invaluable staging of Hodgkin's disease, etc. Most of the British cases never progress beyond stages I or II, even though they may be fatal. For this reason there is much less enthusiasm in this country for a really intensive work-up, at least in the early stages, to include lymphangiography, laparotomy, and splenectomy.
BRITISH MEDICAL JOURNAL
18 AUGUST 1979
With so many doubts about the nature and history of mycosis fungoides, it is hardly surprising that there is no unanimity about treatment. The therapeutic enthusiasts take the line that vigorous early treatment of Hodgkin's disease, some leukaemias, and Burkitt's lymphoma has revolutionised the prognosis of these diseases, and surely the same principle should be applied to mycosis fungoides.1' 12 They advocate full investigation to assess the extent of the disease, followed by energetic treatment of the skin, if this is the only organ diseased. Such treatment includes especially whole-body electron-beam therapy in doses above those normally given for palliative treatment,9 12 or topical mustine applied to the skin regularly for some years.10 11 With the varied history of the disease, we have no convincing evidence that this is justifiable or even kind in most cases, but we need more information. The traditional treatment in Britain is to use purely symptomatic measures in the early stages-emollients, topical or occasionally systemic steroids, and ultraviolet irradiation. When specific infiltrated lesions have appeared various regimens each have their advocates. All are effective for a while, but doubt has been cast whether any really extend the duration as well as quality of life. These measures include radiotherapy with conventional x-rays (60-120 kv) or with electrons, either from a linear accelerator12 13 or from strontium 9014; topical nitrogen mustard; and, most recently, photochemotherapy or PUVA. The results of systemic chemotherapy, using single drugs or quadruple therapy, have been disappointing and this is reserved mainly for cases with systemic disease and a poor prognosis. We have to establish the precise place of photochemotherapy or PUVA therapy in mycosis fungoides,15'l9 as in psoriasis and other benign skin disorders.20 Undoubtedly, by itself it is inadequate for advanced cases of the disease, though it can control the skin changes in early forms of the disease, and may give striking relief of itching. It is too soon yet to know what influence treatment has long term or how often it can achieve complete cure. Relapses tend to occur within at best a few weeks after stopping treatment, but not invariably. At the moment, therefore, it is a compromise between those who wish to give energetic radical treatment to try to eradicate the disease in the early stages and those who hold back active treatment as long as is possible. Which of all of these treatments is used must depend partly on the individual case, but even more on the techniques and experience available in any one centre. Mann, R B, Jaffe, E S, and Berard, C W, American J7ournal of Pathology, 1979, 94, 103. 2 Tan, R S-H, et al, British Journal of Dermatology, 1974, 91, 607. 3 Lutzner, M, et al, Annals of Internal Medicine, 1975, 83, 534. 4 Samman, P D, in Textbook of Dermatology, 3rd edn, ed A J Rook, D S Wilkinson, and F J Ebling, chap 47. Oxford, Blackwell, 1979, in press. 5 Samman, P D, Clinical and Experimental Dermatology, 1976, 1, 197. 6 Winkelmann, R K, Archives of Dermatology, 1973, 108, 205. Prunieras, M, Transactions of the St John's Hospital Dermatological Society, 1975, 61, 1. 8 Rappaport, H, and Thomas, L B, Cancer, 1974, 34, 1198. 9 Levi, J A, and Wiernik, P H, Medicine, 1975, 54, 73. u Van Scott, E J, and Kalmanson, J D, Cancer, 1973, 32, 18. 1 Vonderheid, E C, et al, Archives of Dermatology, 1977, 113, 454. 12 Fuks, Z, and Bagshaw, M A, Radiology, 1971, 100, 145. 13 Spittle, M, Transactions of the St John's Hospital Dermatological Society, 1975, 61, 31. 14 Bratherton, D G, in Modern Trends in Radiotherapy-2, ed T J Deeley, p 176. London, Butterworth. 15 Gilchrest, B A, et al, Cancer, 1975, 38, 683. 16 Hodge, L, et al, British 1977, 2, 1257. Bleehen, S S, Vella Briffa, D, and Warin, A P, Clinical and Experimental Dermatology, 1978, 3, 377. 18 Konrad, K, et al, Hautarzt, 1978, 29, 191. 19 Lowe, N J, et al, Archives of Dermatology, 1979, 115, 50. 20 British 1978, 2, 2.
Medical_Journal,
Medical_Journal,
409
18 AUGUST 1979
rapidly with symptomatic treatment. Physical signs were conspicuously lacking, and no child was detained overnight. During the previous fortnight, the staff recalled, there had been a few cases of a similar type. Some of these children were thought in retrospect to have had attacks of gastroenteritis, for November is the month in which this infection begins to increase in schoolchildren. The next day, 13 more children were affected, and 20 others had recurrences. The staff noticed that while the affected children were being examined other children, hitherto quite well, at once began to complain of symptoms. Meanwhile contaminated food or water, chemical toxins, and common infections were being effectively ruled out as causes. On Friday the school was closed, and no cases were reported from home on that day or at the weekend. Nevertheless, when the school reopened the succeeding Monday 16 new cases and 29 recurrences were recorded, but they proved to be the final cluster of cases. Only a handful more occurred in the rest of the week. In all 196 pupils out of 1663 were affected-18o0 of the girls and 5 o of the boys. The oldest children, aged 10-11, were most affected, and no teachers were ill. Such outbreaks, rare (or at least seldom reported) in the first half of this century, seem lately to have become more frequent. Sirois2 reviewed 20 recorded episodes up to 1973. Many of the school epidemics had occurred in Britain3-6 and in the United States,7-" but schools in Ghana,'2 Malaysia,'3 14 Uganda and Tanzania,'5 and Northern Ireland'6 have also had outbreaks. Similar events have been recorded in children's bands marching, overclad, at football games in the United States'7 18 and in a similar band of young people, including drum majorettes, near Newcastle upon Tyne'9; among nurses at a leprosarium in Japan20; and at factories in the United States2' and in Singapore.22 Many of the school outbreaks have features in common that should suggest a positive diagnosis of epidemic hysteria. Girls make up 70-1000o of those affected; the first cases occur in classes of 12-14 year olds, though younger children may later develop symptoms; the staff and parents or others at home are very seldom ill. The symptoms greatly outweigh any abnormal physical signs, the most common being abdominal pain, faintness, nausea, headache, weakness, and itching. Some girls may be seen to overbreathe. In some cultures more dramatic phenomena occur, such as weeping, wailing, screaming, giggling, running, and claims to have seen ghosts. Spread often seems to be by direct vision: children succumb from seeing other children affected. Another characteristic is that individuals recover very rapidly. The outbreak may, as Sirois says,23 either be explosive (with or without a trigger in the form of one or two mentally or physically sick girls) or slowly cumulative. Sometimes the epidemic may occur in waves, and in day schools very few or no cases occur at intervening weekends. In many outbreaks there has been some cause for underlying anxiety, such as a recent polio epidemic, an unsatisfactory headmaster, impending examinations, or a cultural conflict between home and school. For the school such an epidemic is alarming. The staff may not be aware of the possibility of epidemic hysteria or may be reluctant to entertain the possibility. The search for evidence of infection, especially by viruses, for contamination of food or water, and for toxic gases may take a long time. Meanwhile alarm among parents grows, and the presence of doctors, inspectors, firemen, or police at the school, and reports in the press, extend and prolong the outbreak. Firm reassurance based on a positive diagnosis, without too long a delay to exclude every other conceivable cause, may be very successful.
Failing that, the best policy is that recommended by Johann Weyer24 in the Rhineland in the sixteenth century for dealing with bewitched and demoniacal people in convents: "It is necessary first of all that they all be separated and that each of the girls be sent to her parents or relatives." In other words, for the time being the school should be closed. Figueroa, M, Caribbean Epidemiology Centre Surveillance Report, 1979, 5, No 4. Sirois, F, L'Union Midicale du Canada, 1973, 102, 1906. 3Miller, R, and Raven, M, British Medical_Journal, 1936, 1, 1242. 4Moss, P D, and McEvedy, C P, British Medical_Journal, 1966, 2, 1295. 5 McEvedy, C P, Griffith, A, and Hall, T, British MedicalyJournal, 1966, 2, 1300. 6 Benaim, S, Horder, J, and Anderson, J, Psychological Medicine, 1973, 3, 366. Goldberg, E L, Journal of School Health, 1973, 43, 362. 8 Levine, R J, et al, Lancet, 1974, 2, 1500. 9 Polk, L D, Clinical Pediatrics, 1974, 13, 1013. 10 Nitzkin, J L, Journal of the Florida Medical Association, 1976, 63, 357. "Helvie, C 0,JYournal of School Health, 1968, 38, 505. 12 Adomakoh, C C, Ghana 1973, 12, 407. 13 Teoh, J-I, Singapore Medical3Journal, 1975, 16, 301. 14 Tan, E S, Medical3Journal of Malaya, 1963, 18, 72. 15 Ebrahim, G J, Clinical Pediatrics, 1968, 7, 437. 16 Lyons, H A, and Potter, P E,yournal of the Irish Medical Association, 1970, 63, 377. 17 Pfeiffer, P H, Journal of the Maine Medical Association, 1964, 55, 27. 18 Levine, R J,J_ournal of the American Medical Association, 1977, 238, 2373. 19 Smith, H C T, and Eastham, E J, Lancet, 1973, 2, 956. 20 Ikeda, Y, Psychiatry, 1966, 29, 152. 21 Champion, F P, Taylor, R, Journal of the South Carolina Medical Association, 1963, 59, 351. 22 Chew, P K, Phoon, W H, and Mae-Lim, H A, Singapore Medical3Journal, 1976, 17, 10. 23 Sirois, F, Acta Psychiatrica Scandinavica, 1974, suppl p 252. 24 Quoted in Zilboorg, G (in collaboration with G W Henry), A History of Medical Psychology, p 221. New York, W W Norton, 1941. 2
Medical_Journal,
Mycosis fungoides-unsolved problems What is mycosis fungoides ? The double allusion in the nineteenth century name does not imply any link with a fungus infection. Though generally thought to be a malignant T cell lymphoma arising in the skin, its exact place among the lymphomas is obscure.' One curious feature is epidermotropism-the affinity of its lymphoma cells for the epidermis. Some have also suggested that mycosis fungoides may be a disordered immunological process, a disease of antigen persistence.2 The two concepts are not necessarily mutually completely exclusive. For some investigators mycosis fungoides includes any lymphoma when it affects the skin, for others any lymphoma which seems to arise in the skin. Nevertheless, there is more to be said for restricting the term to an entity characterised by its clinical picture and by the distribution, cytology, and ultrastructure3 of the cellular infiltrate in the skin. Unfortunately, these various criteria may not all be satisfied in every case; and no single one seems invariable. For example, the histological appearances of a very aggressive lymphoma type may be associated with an apparently good prognosis in lymphomatoid papulosis, and cells with the ultrastructural appearances of those in mycosis fungoides may be found in benign inflammatory dermatoses. Classical mycosis fungoides4 5 starts with a rather characteristic eruption of fixed erythematous plaques, which are not apparently grossly infiltrated and are devoid of the more florid histological changes found later in the disease. After months, years, or decades the more characteristic infiltrated plaques and even
410
tumours may arise, and may lead on within a year or two to death from sepsis or from systemic disease. Clinical confusion arises about the so-called premycotic eruptions, and also around the "d'emblee" forms of the disease, which do not show the early phase. The term parapsoriasis is hallowed by tradition, but unless qualified it is best avoided. Undoubtedly many patients develop fixed erythematous lesions which may not clear but never develop into mycosis fungoides. This eruption is sometimes called benign parapsoriasis or, perhaps better, persistent superficial dermatitis.4 Other cases are superficially rather similar, but closer inspection by an experienced eye shows some of the tell-tale signs of true mycosis fungoides: the varying hue of the lesions, the presence of atrophy, and telangiectasia, even amounting to poikiloderma. This type is sometimes given the name premycotic type of parapsoriasis. Nevertheless, this is perhaps a misnomer because it seems likely that the eruption has been mycosis fungoides from the beginning.4 The distinction between the two processes may be extremely difficult clinically. The presence or absence of itching is not a reliable guide. Doubt often remains even after repeated examinations and several biopsies. But this is not equivalent to saying that the diseases really overlap or that the persistent superficial dermatitis can really evolve into mycosis fungoides, though the possibility has not been excluded. It more reflects our lack of adequate diagnostic criteria. The nature of the d'emblee cases is also in doubt. These start with infiltrated plaques or tumours and run their course more rapidly. Histologically these may be indistinguishable from classical mycosis fungoides but some such patients have B-cell lymphomas rather than the T-cell proliferation of mycosis fungoides and deserve to be kept apart. Likewise patients who present with an erythroderma deserve to be separated off. Some are examples of the Sezary syndrome with its distinctive clinical picture and characteristic "cellules monstreuses" in the peripheral blood. The relationship of the Sezary syndrome to mycosis fungoides, of which it may be a leukaemic variant, is undecided.6 7 The history of classical mycosis fungoides is extremely variable, and the prognosis, at least in the early stages, is far from gloomy, though it may spoil life without destroying it. In many cases the "premycotic" eruption may never evolve into the more specific infiltrative phase, even after 50 years. In Samman's British series5 only 8% of patients had died ofthe disease within 10 years of onset, though once the infiltrated plaques and tumours have arisen the prognosis is far from good. The presence of enlarged lymph nodes, whether from specific infiltration or just from inflammatory changes, adversely affects the prognosis. In Britain deaths from the disease are usually due to secondary infection, and a distressing end state this may be; systemic disease, with the possible exception of spread to the lymph nodes, is rare. Interestingly, figures from the United States give a different emphasis.8 9 There systemic disease is much more common. This may reflect a different pattern of referral to the major centres, or be due to a true heterogeneity of the disease itself. Quite possibly more than one disease process is masquerading under the clinical picture of classical mycosis fungoides. Various criteria for staging of mycosis fungoides have been published'° 11 comparable with the invaluable staging of Hodgkin's disease, etc. Most of the British cases never progress beyond stages I or II, even though they may be fatal. For this reason there is much less enthusiasm in this country for a really intensive work-up, at least in the early stages, to include lymphangiography, laparotomy, and splenectomy.
BRITISH MEDICAL JOURNAL
18 AUGUST 1979
With so many doubts about the nature and history of mycosis fungoides, it is hardly surprising that there is no unanimity about treatment. The therapeutic enthusiasts take the line that vigorous early treatment of Hodgkin's disease, some leukaemias, and Burkitt's lymphoma has revolutionised the prognosis of these diseases, and surely the same principle should be applied to mycosis fungoides.1' 12 They advocate full investigation to assess the extent of the disease, followed by energetic treatment of the skin, if this is the only organ diseased. Such treatment includes especially whole-body electron-beam therapy in doses above those normally given for palliative treatment,9 12 or topical mustine applied to the skin regularly for some years.10 11 With the varied history of the disease, we have no convincing evidence that this is justifiable or even kind in most cases, but we need more information. The traditional treatment in Britain is to use purely symptomatic measures in the early stages-emollients, topical or occasionally systemic steroids, and ultraviolet irradiation. When specific infiltrated lesions have appeared various regimens each have their advocates. All are effective for a while, but doubt has been cast whether any really extend the duration as well as quality of life. These measures include radiotherapy with conventional x-rays (60-120 kv) or with electrons, either from a linear accelerator12 13 or from strontium 9014; topical nitrogen mustard; and, most recently, photochemotherapy or PUVA. The results of systemic chemotherapy, using single drugs or quadruple therapy, have been disappointing and this is reserved mainly for cases with systemic disease and a poor prognosis. We have to establish the precise place of photochemotherapy or PUVA therapy in mycosis fungoides,15'l9 as in psoriasis and other benign skin disorders.20 Undoubtedly, by itself it is inadequate for advanced cases of the disease, though it can control the skin changes in early forms of the disease, and may give striking relief of itching. It is too soon yet to know what influence treatment has long term or how often it can achieve complete cure. Relapses tend to occur within at best a few weeks after stopping treatment, but not invariably. At the moment, therefore, it is a compromise between those who wish to give energetic radical treatment to try to eradicate the disease in the early stages and those who hold back active treatment as long as is possible. Which of all of these treatments is used must depend partly on the individual case, but even more on the techniques and experience available in any one centre. Mann, R B, Jaffe, E S, and Berard, C W, American J7ournal of Pathology, 1979, 94, 103. 2 Tan, R S-H, et al, British Journal of Dermatology, 1974, 91, 607. 3 Lutzner, M, et al, Annals of Internal Medicine, 1975, 83, 534. 4 Samman, P D, in Textbook of Dermatology, 3rd edn, ed A J Rook, D S Wilkinson, and F J Ebling, chap 47. Oxford, Blackwell, 1979, in press. 5 Samman, P D, Clinical and Experimental Dermatology, 1976, 1, 197. 6 Winkelmann, R K, Archives of Dermatology, 1973, 108, 205. Prunieras, M, Transactions of the St John's Hospital Dermatological Society, 1975, 61, 1. 8 Rappaport, H, and Thomas, L B, Cancer, 1974, 34, 1198. 9 Levi, J A, and Wiernik, P H, Medicine, 1975, 54, 73. u Van Scott, E J, and Kalmanson, J D, Cancer, 1973, 32, 18. 1 Vonderheid, E C, et al, Archives of Dermatology, 1977, 113, 454. 12 Fuks, Z, and Bagshaw, M A, Radiology, 1971, 100, 145. 13 Spittle, M, Transactions of the St John's Hospital Dermatological Society, 1975, 61, 31. 14 Bratherton, D G, in Modern Trends in Radiotherapy-2, ed T J Deeley, p 176. London, Butterworth. 15 Gilchrest, B A, et al, Cancer, 1975, 38, 683. 16 Hodge, L, et al, British 1977, 2, 1257. Bleehen, S S, Vella Briffa, D, and Warin, A P, Clinical and Experimental Dermatology, 1978, 3, 377. 18 Konrad, K, et al, Hautarzt, 1978, 29, 191. 19 Lowe, N J, et al, Archives of Dermatology, 1979, 115, 50. 20 British 1978, 2, 2.
Medical_Journal,
Medical_Journal,
![[Superficial mycosis].](/assets/img/hold.png)
