Generalized Pustularpsoriasis

12 July 1969 Leading Articles ,,B, 71 the facts in any specific case. of Baker and Ryan's patients the preceding psoriasis had but it is necessary to consider Br Med J: first published as 10.1136/bmj.3.5662.71 on 12 July 1969. Downloaded from In those cited large amounts of arsenic had contaminated the been in some way atypical and of short duration. water from an industrial source. Generalized pustular psoriasis may occur with no history of Any arsenic in surface water is likely to be in insoluble preceding skin disease and is then indistinguishable from suspended matter, and this can be readily removed by coagula- what used to be called impetigo herpetiformis. Attacks of tion and filtration as normally practised in waterworks. If pustulation last only a few days or weeks, during which time the water loses its oxygen content through prolonged contact the patient may be desperately ill with high fever and leuco- with decomposing organic matter-for instance, at the bottom cytosis, and a fatal outcome is not uncommon. As the of a reservoir in which water circulation is insufficient- pustulation settles down the skin either returns to normal or insoluble arsenic could theoretically be slowly and partially else a psoriatic erythroderma supervenes and may persist for converted to soluble, and this would need much higher doses many months or even years. of coagulants for removal. But there is no record of this The aetiology of all forms of psoriasis is still uncertain, effect having occurred, and if the bottom water is periodically but it is usually accepted as a genetically determined abnor- or continuously drained away, as it would be from this mality which may be brought to light by a variety of stimuli, reservoir, the question should not arise. including, for example, streptococcal sore throats and None of numerous analyses of the river water in the neigh- emotional stress. Often no precipitating factor is detected. bourhood of the proposed dam has approached the W.H.O. Likewise a trigger factor for pustular psoriasis should be maximum allowable limit even before simple filtration treat- sought, though once the disease process is under way ment, and all have been free from detectable arsenic (0 005 removal of the initiating cause may have little effect on its mg./l.) after such treatment. There may be good reasons subsequent course. Infections, stress, pregnancy, and hypo- for opposing the construction of a reservoir on the West calcaemia have all been held responsible. Reactions to Okement river, but there are no grounds for suggesting that various drugs, including antimalarials, iodides, and sali- the water may be dangerous. cylates, have been reported. The most important precipitating factor is the previous administration of systemic corticosteroids for ordinary psoriasis, a practice now almost universally condemned. Baker and Ryan attributed at least 21 of their 104 cases to this cause, attacks usually occurring soon after withdrawal Generalized Pustular Psoriasis of the drugs. In two cases intensive topical therapy with In most cases psoriasis is a mild disease, seldom of more than corticosteroid ointments under polyethylene occlusion seemed cosmetic significance. But the most severe cases are incapaci- to be responsible. This danger, combined with the fact that tating and even endanger life. Generalized pustular psoriasis the remissions which follow the use of corticosteroid oint- can at is ments are shorter than those achieved with conventional tar be considered as psoriasis its most severe. It fortu- http://www.bmj.com/ nately rare, but there has probably been some increase in its or dithranol preparations, is good reason for not relying on incidence in the past few years owing partly to over- such treatment, despite its obvious advantages of a quick enthusiastic treatment. Previous reports have considered initial response and relative lack of mess, as the mainstay small numbers of cases, but H. Baker and T. J. Ryan1 2 have in routine treatment of psoriasis. now collected together details of 104 cases-24 from Treatment of generalized pustular psoriasis is a very St. John's Hospital for Diseases of the Skin and the difficult problem.2 Both systemic corticosteroids, sometimes remainder from dermatologists throughout the country. in large doses, and methotrexate will control the pustulation, Although bacteria may be found in unusual numbers but there is no evidence that they improve the long-term on 25 September 2021 by guest. Protected copyright. colonizing psoriatic skin, actual infection, with pustule prognosis and considerable evidence that they make it worse. formation, is uncommon. The pustules of pustular psoriasis Once started, it is all too difficult to leave off systemic are generally sterile. The chemical changes in psoriatic skin corticosteroids, and larger and larger doses may be required which lead to the immigration to it of polymorphonuclear to achieve less and less effect, until the morbidity and leucocytes have not yet been defined.3 Histologically minute mortality of the treatment are much greater than those of abscesses are frequently found in the epidermis in ordinary the disease. Methotrexate also has its own severe toxic psoriasis, but they remain microscopic in size. Plaques of side-effects, usually apparent at an earlier stage. The con- chronic psoriasis at times show macroscopic, sterile pustules, clusion is that both drugs should be withheld if possible to especially at times of increased activity of the disease or after allow the disease to run its own usually self-limiting course, irritant local therapy. the physician being prepared to wait until at least three Pustular psoriasis on the palms and soles is a relatively months' hospital treatment with bland local applications has common disease,4 but is only rarely followed by pustulation failed. Conventional topical applications for psoriasis such elsewhere. There are several distinct but overlapping as tar and dithranol are contraindicated. patterns of true generalized pustular psoriasis, all charac- The rare patients who are in immediate danger of dying terized by the development of sheets of small sterile pustules in the acute phase, those who continue to get active pustu- just beneath the horny layer of the epidermis, often super- lation for many weeks on end, and those who are left with imposed on a diffuse bright red erythema. The whole an intractable psoriatic erythroderma may demand more picture does not obviously resemble the familiar pattern of active treatment as a life-saving measure. For these the chronic psoriasis. This eruption may cover limited areas of the body, the flexural more often than the extensor aspects, 1 Baker, H., and Ryan, T. J., British 7ournal of Dermatology, 1968, 80, or it may be almost universal, even involving the mucous 2Ryan, T. J., and Baker, H.,BrItish 7ourna of Dermatology, 1969, 81, 134. membranes. Attacks of pustulation may occur in patients 'Langhof, H., and Muller, H., Hautarzt, 1966 17, 101. with long-standing ordinary psoriasis and are then often 4Ashurst, P. J. C., British 7ournal of Dermatol'ogy, 1964, 76, 169. 5Zumbusch, L. R. von, Archiv fur Dermnatologie und Syphilis, 1910, provoked by some stimulus which is apparent, but in 40% 99, 335. 72 12 July 1969 Leadig Articles MEDICALB11RisiCOJOURNAL is patients, but there was failure in the sixth because of unsuit- evidence insufficient to show whether corticosteroids or Br Med J: first published as 10.1136/bmj.3.5662.71 on 12 July 1969. Downloaded from methotrexate are to be preferred, but slightly favours the able shape and size of the electrode. latter. There is much to be said for not prolonging treatment This simple technique is likely to have increasing applica- with these agents or for using heroic doses to try to achieve a tion in the control of anal incontinence. For some patients it complete remission. It is better to restrict treatment to the will restore sphincter control ; for others it will make life control of the immediate life-threatening situation. more tolerable. The original case reported in 1910 by von Zumbusch,5 whose name is usually linked with this disease, survived nine attacks of generalized pustular psoriasis of decreasing severity. Whether this would have been the outcome had modern methods of treatment been available is of Junior Doctors problematical. Representation In losing a battle the hospital junior doctors have gained sympathy for their cause. As reported in the Supplement, the Representative Body at Aberdeen was asked to create a standing committee of the Association for hospital junior staff to, among other things, dispel the belief that their Faecal Incontinence interests are neglected by the Central Committee for Hospital Medical Services. Several speakers emphasized how wide- One of the most distressing of disabilities is anal incontinence. spread is this view among junior medical staff, and pressed Among its many causes may be specially noted the scarring that the Association should destroy the myth by showing its of an extensive perineal tear in childbirth, ill-contrived anal willingness to meet their requests. For most the plea was not surgery, spinal cord trauma or disease, and severe rectal pro- that justice was not being done ; it was that it should obvi- lapse, and may or may not be complicated by urinary ously be seen to be done. Agreement to a standing committee incontinence. The patient, often an elderly woman, becomes for hospital junior staff was described by Dr. J. F. G. Pigott, cut off from family and friends, a nuisance in an old people's chairman of the Junior Hospital Doctors' Association, as the home or hospital for patients with chronic diseases, and more one remaining ground of conflict between the J.H.D.A. and liable than others to develop pressure sores. the B.M.A. Until recently little could be offered to many of these Many of the 14,000 junior doctors can rightly point to patients, but K.

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