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A.C.T.H. IN ERYTHEMA MULTIFORME EXUDATIVUM MAJOR (STEVENS-JOHNSON SYNDROME)
875 surgeon, his retort was : Fear not, for the ba,rber surgeons cannot even read French." The type of cryptogram first introduced by Leonardo has a subsequent parallel. In the archives of the Cavendish Laboratories at Cambridge, there are preserved some letters by the famous mathematician Clerk Maxwell, in which he writes many of his English words using the Greek hieroglyphics-a manoeuvre I have on occasion used in the past in my hospital outpatient case-records to
preserve my unpalatable diagnoses from the
eye of the
vzvpaa8zvLx patient.
prying
N. ASHERSON.
London, W.I.
A.C.T.H. IN ERYTHEMA MULTIFORME EXUDATIVUM MAJOR (STEVENS-JOHNSON SYNDROME)
SiR,-The aetiology of erythema multiforme exudatiremains obscure and treatment largely unsatisfactory. Recent publications have dealt with the history, and aetiology of the condition.!2 The nomenclature, " Stevens-Johnson name syndrome has been "suggested for the major or severe form, and the sunix Hebra " for the mild form without stomatitis and ophthalmia. Efforts to find a virus or other infectious agent have been inconclusive or negative. A virus has been sought without success in the usual laboratory animals and in horses, cows, sheep, pigs, and embryonated eggs.34 In 1950 we saw an 8-month-old boy with characteristic erythema multiforme exudativum major, with polymorphous rash, oral ulceration, conjunctivitis, keratitis, balanitis, and extensive pneumonitis. He had become progressively worse during 2 months’ treatment with vum
"
antibiotics, sulphonamides, blood-transfusions, and dietary alterations.
premise that sensitisation may in play important part the evolution of this syndrome, he was given adrenocorticotropic hormone (A.C.T.H.) (Armour). There was a striking response within 4 days, and the lesions began to clear up. The illness began on Aug. 30, 1950, with cough and rhinitis, succeeded in a few days by vesicular lesions in the mouth and on the scrotum, followed shortly by conjunctivitis,
variety of infectious diseases, but none simulating the complete picture of erythema multiforme exudativum major. Such rashes are generally ascribed to an allergic response to the infecting organism. One of us has seen a child who, during the course of acute primary herpetic gingivostomatitis in which the herpes virus was isolated and there was a rise in the specific antibody titre, developed erythema multiforme of the more usual type, as seen occasionally during the course of various infections. The picture could scarcely be confused with erythema multiforme exudativum major, despite the gingivostomatitis. We think that the aetiology of this condition is multiple, but that sensitisation is important. Under such circumIn stances A.C.T.H. or cortisone should be effective. the presence of mucosal lesions of this type antibiotics orally may be ineffective and even risky.5 ALEX J. STEIGMAN STEICtMAN LOuIsvIlle, University of Louisville, J J. J. J KELLY. K Kentucky. TREATMENT OF SCARLET FEVER SiR,-It appears that in 1950 there were 65,883 cases of scarlet fever notified in England and ’Vales, compared with 56,851 cases in the whole of the United States. A World Health Organisation survey 6 concludes that " Europe is still the world epidemic centre " for scarlet fever ; but I believe that these figures may more truly represent the enlightenment on the other side of the Atlantic in making no diagnostic distinction between streptococcal throat infections accompanied by a rash and those not so accompanied.
On the
an
keratitis, irregular fever, rapid loss of weight, and radiographic signs of scattered density in both lungs. Before having A.C.T.H. he received sulphadiazine, penicillin, streptomycin,
aureomycin, chloramphenicol, repeated blood-transfusions, and soya-bean and casein digest substitutes instead of a cow’s milk diet. Fever was irregular, weight loss progressive, and pulmonary signs, both clinical and radiographic, continued Local therapy (antibiotics, gentian-violet, for 2 months. drying lotions) did not modify the course of the illness. Bacteriological examination of the blood, saliva, urine, The bone-marrow was and ocular exudate was negative. normal; red-cell counts showed a mild hypochromic anaemia ; white-cell counts ranged from 10,500 to 27,200 in ten counts, and no eosinophils were seen except on Sept. 19 when they numbered 5% of a total of 14,000 cells. On Oct. 25 he was given 20 mg. of A.C.T.H. every 6 hours. Within 4 days the oral and ocular lesions began to resolve. A radiograph of the chest taken after 5 days’ treatment with A.C.T.H. showed a remarkable change. A film taken just before treatment showed " involvement of all lobes, the uppers most severely ; consistent with a clinical diagnosis of fungus infection." The next film showed" marked improvement and resolution in all involved areas." The dosage of A.C.T.H. was reduced to 15 mg. every 6 hours on Oct. 30 and to 10 mg. every 8 hours on Nov. 6. It was stopped altogether on Nov. 13. A follow-up note and photograph one year later indicated normal growth and development with no recurrence of symptoms or signs.
A considerable effort has been made to find an infectious agent responsible for this syndrome. Maculopapular forms of erythema multiforme have been observed in a 1. Ashby, D. W., Lazar, T. Lancet, 1951, i, 1091. 2. J. Amer. med. Ass. 1951, 146, 1424. 3. Commission on Acute Respiratory Diseases. Arch. intern. Med. 1946, 78, 687. 4. Anderson, J. A., Bolin, V. Arch. Derm. Syph., Chicago, 1949, 59, 251
C. GLAISHER.
Hildenborough, Kent.
AN UNUSUAL EPIDEMIC
SIR,-I have been extremely interested by the many letters reporting cases of an unusual epidemic." This illness is apparently not limited to England ; and in the past two months I have seen about 35 such cases, which I am tempted to call encephalitis. "
is variable, but the most prevalent headache and vertigo. The headache is not very acute, and in many cases it is more a vague feeling of heaviness. Often the patients complain of pain at the back of the head and in the neck, and pressure on the point of emergence of the great occipital nerve is generally painful. Vertigo is commonly also present, though it may be lacking ; in my experience it is generally mild, however long it may The clinical
symptoms
picture
are
last. A few patients have complained of temporary impairment of their mental faculties. A woman told me that, having to add up a few figures, she said : "five and five make thirteen " ; and a man was worried because he could not write an ordinary letter without making a mistake every other line. In no case was the headache strong enough to explain per se these other troubles. Occasionally I have noticed stiffness of the neck and-especially in children-
positive Kernig’s sign. In one case only warrant a lumbar was almost normal.
were
the
puncture,
symptoms and
the
severe enough to cerebrospinal fluid
Curiously enough, for contagious, I have never
a disease which seems to be met two cases of it in the same household. This may be explained by the existence of subclinical forms, as I heard of people having suffered from headache for a day or two only. Intravenous injections of sodium salicylate may not be entirely devoid of effect ; but aureomycin seems specially useful. A large number of patients quickly recovered after 2 g. a day for 4-5 days, whilst untreated cases may apparently last 2-3 months. The impression is that this is a viral disease. Whether all the cases reported in your columns are of the same disease, and whether the epidemic I have seen in
5. Lancet, 1951, ii, 532. 6. See Times, 19, 1952.
April
preserve my unpalatable diagnoses from the
eye of the
vzvpaa8zvLx patient.
prying
N. ASHERSON.
London, W.I.
A.C.T.H. IN ERYTHEMA MULTIFORME EXUDATIVUM MAJOR (STEVENS-JOHNSON SYNDROME)
SiR,-The aetiology of erythema multiforme exudatiremains obscure and treatment largely unsatisfactory. Recent publications have dealt with the history, and aetiology of the condition.!2 The nomenclature, " Stevens-Johnson name syndrome has been "suggested for the major or severe form, and the sunix Hebra " for the mild form without stomatitis and ophthalmia. Efforts to find a virus or other infectious agent have been inconclusive or negative. A virus has been sought without success in the usual laboratory animals and in horses, cows, sheep, pigs, and embryonated eggs.34 In 1950 we saw an 8-month-old boy with characteristic erythema multiforme exudativum major, with polymorphous rash, oral ulceration, conjunctivitis, keratitis, balanitis, and extensive pneumonitis. He had become progressively worse during 2 months’ treatment with vum
"
antibiotics, sulphonamides, blood-transfusions, and dietary alterations.
premise that sensitisation may in play important part the evolution of this syndrome, he was given adrenocorticotropic hormone (A.C.T.H.) (Armour). There was a striking response within 4 days, and the lesions began to clear up. The illness began on Aug. 30, 1950, with cough and rhinitis, succeeded in a few days by vesicular lesions in the mouth and on the scrotum, followed shortly by conjunctivitis,
variety of infectious diseases, but none simulating the complete picture of erythema multiforme exudativum major. Such rashes are generally ascribed to an allergic response to the infecting organism. One of us has seen a child who, during the course of acute primary herpetic gingivostomatitis in which the herpes virus was isolated and there was a rise in the specific antibody titre, developed erythema multiforme of the more usual type, as seen occasionally during the course of various infections. The picture could scarcely be confused with erythema multiforme exudativum major, despite the gingivostomatitis. We think that the aetiology of this condition is multiple, but that sensitisation is important. Under such circumIn stances A.C.T.H. or cortisone should be effective. the presence of mucosal lesions of this type antibiotics orally may be ineffective and even risky.5 ALEX J. STEIGMAN STEICtMAN LOuIsvIlle, University of Louisville, J J. J. J KELLY. K Kentucky. TREATMENT OF SCARLET FEVER SiR,-It appears that in 1950 there were 65,883 cases of scarlet fever notified in England and ’Vales, compared with 56,851 cases in the whole of the United States. A World Health Organisation survey 6 concludes that " Europe is still the world epidemic centre " for scarlet fever ; but I believe that these figures may more truly represent the enlightenment on the other side of the Atlantic in making no diagnostic distinction between streptococcal throat infections accompanied by a rash and those not so accompanied.
On the
an
keratitis, irregular fever, rapid loss of weight, and radiographic signs of scattered density in both lungs. Before having A.C.T.H. he received sulphadiazine, penicillin, streptomycin,
aureomycin, chloramphenicol, repeated blood-transfusions, and soya-bean and casein digest substitutes instead of a cow’s milk diet. Fever was irregular, weight loss progressive, and pulmonary signs, both clinical and radiographic, continued Local therapy (antibiotics, gentian-violet, for 2 months. drying lotions) did not modify the course of the illness. Bacteriological examination of the blood, saliva, urine, The bone-marrow was and ocular exudate was negative. normal; red-cell counts showed a mild hypochromic anaemia ; white-cell counts ranged from 10,500 to 27,200 in ten counts, and no eosinophils were seen except on Sept. 19 when they numbered 5% of a total of 14,000 cells. On Oct. 25 he was given 20 mg. of A.C.T.H. every 6 hours. Within 4 days the oral and ocular lesions began to resolve. A radiograph of the chest taken after 5 days’ treatment with A.C.T.H. showed a remarkable change. A film taken just before treatment showed " involvement of all lobes, the uppers most severely ; consistent with a clinical diagnosis of fungus infection." The next film showed" marked improvement and resolution in all involved areas." The dosage of A.C.T.H. was reduced to 15 mg. every 6 hours on Oct. 30 and to 10 mg. every 8 hours on Nov. 6. It was stopped altogether on Nov. 13. A follow-up note and photograph one year later indicated normal growth and development with no recurrence of symptoms or signs.
A considerable effort has been made to find an infectious agent responsible for this syndrome. Maculopapular forms of erythema multiforme have been observed in a 1. Ashby, D. W., Lazar, T. Lancet, 1951, i, 1091. 2. J. Amer. med. Ass. 1951, 146, 1424. 3. Commission on Acute Respiratory Diseases. Arch. intern. Med. 1946, 78, 687. 4. Anderson, J. A., Bolin, V. Arch. Derm. Syph., Chicago, 1949, 59, 251
C. GLAISHER.
Hildenborough, Kent.
AN UNUSUAL EPIDEMIC
SIR,-I have been extremely interested by the many letters reporting cases of an unusual epidemic." This illness is apparently not limited to England ; and in the past two months I have seen about 35 such cases, which I am tempted to call encephalitis. "
is variable, but the most prevalent headache and vertigo. The headache is not very acute, and in many cases it is more a vague feeling of heaviness. Often the patients complain of pain at the back of the head and in the neck, and pressure on the point of emergence of the great occipital nerve is generally painful. Vertigo is commonly also present, though it may be lacking ; in my experience it is generally mild, however long it may The clinical
symptoms
picture
are
last. A few patients have complained of temporary impairment of their mental faculties. A woman told me that, having to add up a few figures, she said : "five and five make thirteen " ; and a man was worried because he could not write an ordinary letter without making a mistake every other line. In no case was the headache strong enough to explain per se these other troubles. Occasionally I have noticed stiffness of the neck and-especially in children-
positive Kernig’s sign. In one case only warrant a lumbar was almost normal.
were
the
puncture,
symptoms and
the
severe enough to cerebrospinal fluid
Curiously enough, for contagious, I have never
a disease which seems to be met two cases of it in the same household. This may be explained by the existence of subclinical forms, as I heard of people having suffered from headache for a day or two only. Intravenous injections of sodium salicylate may not be entirely devoid of effect ; but aureomycin seems specially useful. A large number of patients quickly recovered after 2 g. a day for 4-5 days, whilst untreated cases may apparently last 2-3 months. The impression is that this is a viral disease. Whether all the cases reported in your columns are of the same disease, and whether the epidemic I have seen in
5. Lancet, 1951, ii, 532. 6. See Times, 19, 1952.
April