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SELECTIVE IgA DEFICIENCY AND IDIOPATHIC THROMBOCYTOPENIC PURPURA
861
knowledge, West et al.’ were the first to report selective IgA deficiency in patients with diseases of central nervous system, including epilepsy. Since we reported selective IgA deficiency in a patient with postencephalitic epilepsy in 1968we have paid special attention to the effect of anticonvulsants, es-
PHENYTOIN IN BENIGN INSULINOMA
our
pecially phenytoin, on IgA, and we have seen twenty-two patients (aged 7-56 years) of whom eight had epilepsy. Six of the patients were on phenytoin alone or in combination with phenobarbitone, one was on carbamazepine alone, and one was being treated with trimetadione and phenobarbititone. In addition, we found primary acquired agammaglobutinsemia (one case) and type-i dysgammagtobulinaemia (one case) in patients with epilepsy receiving phenytoin; these patients lacked both serum and secretory IgA. Two of the patients with selective IgA deficiency and epilepsy had recurrent repiratory infections, and two had recurrent fevers. In the case of agammaglobulinxmia, pneumonia and acute otitis media
common, whereas the patient with had recurrent attacks of pustular dysgammaglobulinaemia lesions, usually limited to the hands. Immunohaematological abnormalities are common in patients with selective IgA deficiency and in patients on anticonvulsants. One patient with selective IgA deficiency and epilepsy had systemic lupus erythematosus.7 In selective IgA deficiency circulating IgA-bearing B lymphocytes are usually normal but we have seen a case with fewer than 1% of IgA-bearing B lymphocytes.’ Antinuclear antibodies and anti-IgA antibodies were positive in some patients with immunodeficiency diseases and epilepsy, and of thirty-five epileptics treated with phenytoin, three had antinuclear antibodies and seven had anti-IgA antibodies.8 Moreover, in one patient an abnormal serum-IgA (5 mg/dl) rose to 20 mg/dl when phenytoin was withdrawn, in parallel with a rise in the numbers of IgA-containing plasma-cells in the jejunal mucosa. This case suggests that IgA deficiency in patients receiving phenytoin is not always irreversible.9 Two other non-epileptic patients whose serum-IgA became undetectable had autoimmune diseases-namely, systemic lupus erythematosus and periarteritis nodosa.OJ We conclude that: (1) Phenytoin can mainly affect the IgA system, but sometimes other immunoglobulins also. 10 (2) Selective IgA deficiency may be induced11by the drug through the development of autoimmunity. Besides the defective secretion or increased catabolism of IgA, the suppression of lymphoid tissue by anti-IgA antibodies may be implicated in the pathogenesis of13selective IgA deficiency, as shown in animal experiments. 12 (3) Phenytoin may directly affect IgA-producing cells in the gut-associated lymphoid tissues. (4) Autoimmunity, induced by the drug could occur in conjunction with immunodeficiency diseases. Anticonvulsants, mainly phenytoin, 8 have been linked with a broader spectrum of immunopathies, including lymphoma. These findings would be pertinent to the pathogenesis of malignant lymphomas, which also can be induced by the drug."I were
First Division, Department of Internal Medicine,
Kyoto University, Kyoto 606, Japan.
T. KANOH H. UCHINO
5. West, C. D., Hong, R., Holland, N. H. J. clin. Invest. 1962, 41, 2054. 6. Kanoh, T., Wakisaka, G. Acta hâem. jap. 1968, 31, 481. 7. Takigawa, M., Kanoh, T., Imamura, S., Takahashi, C. Archs Derm. (in the press). 8 Kanoh, T., Yamaguchi, N. Excerpta med. int. Congr. Ser. 1973, no. 300, p. 71. 9. Kanoh, T., Niwa, Y., Yamaguchi, N. J. Jap. Soc. intern. Med. 1975, 64, 1353. 10 Grob, P. J., Herold, G. E. Br. med. J. 1972, ii, 561. 11. Kanoh, T. Acta hâem. jap. 1971, 34, 452. 12 Manning, D. D. J. Immun. 1972, 109, 1152. 13 Sorrell, T. C., Forbes, I. J., Burness, F. R., Rischbirth, R. H. C. Lancet,
1971, ii, 1233.
(diphenylhydantoin) inhibits insulin blood-levels similar to those effective in treating epilepsy.12 It inhibits insulin release from the labile and the large storage pools of insulin in the B cells. We have studied the effect of phenytoin on glucose and insulin concentrations in a 32-year-old man with symptoms of insulin excess. Laparotomy revealed a benign 2 x2 cm insulinoma in the head of the panSIR,-Phenytoin
release
at
creas.
Before operation, a prolonged fasting test was carried out and was repeated on the third day of oral phenytoin treatment (given in divided doses, 600 mg daily for two days and 300 mg on the third day). Serum-glucose and plasma-immunoreactiveinsulin (I.R.I.) were measured every 6 h: Time
Glucose
I.R.I.
(h offasting) Before phenytozn:
(mg/d/)
([LU/Ml)
12 18 24 28
74 50 12 22
8.0 6.0 6.0 6.0
Mean±S.E.M.
39-5±14.0
6.5:t0.5
60 60 29 40
4.0 4.0 4.0 3.0
15.0 15.0 7.25
47.3:t13.3
3.7:t0.25
12.6:t1.84
Glucose/I.R.I. 9.25 8.33 2.00 3.66
5.81:t1.75
After phenytoin: 12 18 24 28 Mean+S.E.M.
13.3
During phenytoin therapy, there was a significant rise in the
glucose/!.R.I. ratio although the change in glucose was not significant. Although there was a significant fall in plasma-i.R.I., the baseline was not high, suggesting that the tumour was secreting principally proinsulin.3 The effects of acute phenytoin treatment are similar to those previously reported .4 5 Future studies on the long-term effects of this treatment, especially in patients with unresectable tumours, would be of interest. Department of Internal Medicine, American University of Beirut Hospital, Beirut, Lebanon
MOH’D AMIN ARNAOUT IBRAHIM SALTI
SELECTIVE IgA DEFICIENCY AND IDIOPATHIC THROMBOCYTOPENIC PURPURA
SiR,—You have lately reviewed6
new
concepts in selective
IgA deficiency. The development in these patients of a variety of autoimmune disorders is established, but only one case of idiopathic thrombocytopenic purpura (I. ToP.) has been noted.7 We wish to report two such instances. Case 1.-A 19-year-old male was referred with a 1-year history of easy bruising. Slight splenomegaly was found, and I. T.P. was diagnosed. IgA was undetectable by radial immunodiffusion, the IgM level was normal; a polyclonal increase of IgG (2 g/dl) was noted. There was no pertinent past history.
Familial studies including immunoglobulin levels were unremarkable. Coombs test, antinuclear factors, and rheumatoid factors were negative. T-cell function was normal. After an unsuccessful course of steroids, the spleen was removed and the platelet-count returned to normal. Case 2.-A 40-year-old woman had had, for the past 5 years, mild sinusitis, tonsillitis, and recurrent urinary-tract infections. The findings of severe thrombocytopenia led to further evaluation. There was a slight splenomegaly and a mild Raynaud’s phenomenon. She had no IgA, with a poly1. Malherbe, C., Burril, K. C., Levin, S. R., Karam, J. H., Forsham, P. H. New
Engl. J. Med. 1972, 286, 339. 2.
Levin, S. R., Grodsky, G. M., Hagura, R., Smith, D. Diabetes, 1972, 21,
856. 3. Sherman, B. M., Pek, E., Fajans, S. S., Floyd, J. C., Conn, J. W. J. clin. Endocr. Metab. 1972, 35, 271. 4. Cotten, M. S., Bower, R. H., Fidler, S. M., Johnsonbaugh, R. E., Sode, J. Lancet, 1973, i, 40. 5. Pelkoner, R., Taskinen, M. R. ibid. p. 604. 6. Lancet, 1975, ii, 1291. 7. Hong, R., Ammann, A. J. Am. J. Path. 1972, 69, 491.
862
IgM and IgG (2-5 g/dl); a circulating anticephalin anticoagulant; and positive Coombs test with elution of an IgG anti-e antibody without overt haemolysis during a 2-year period. It is thus probable that I.T.P. may be associated with IgA deficiency although the true incidence of this disorder in I.T.P.
clonal increase of
,
to
Laboratory of Immunochemistry (INSERM Research Institute on Blood
Diseases,
Hôpital Saint-Louis, 75475 Paris Cedex 10, France
U
108)
JEAN-CLAUDE BROUET MAXIME SELIGMANN
POLYCYSTIC RENAL DISEASE, HYPERTENSION, AND IgA DEFICIENCY
SIR,-Dr Gabriel (Jan. 24, p. 197) raises the possibility that hypertension, multicystic disease of the kidney, and IgA deficiency might not be a fortuitous combination. The following case
patients develop
stages.’
be established. Since a subtle T-cell defect has been postulated in IgA deficiency it is advisable to evaluate carefully T-cell function in such patients when splenectomy is recommended. Low levels of IgA or selective IgA deficiency have been previously found in patients with autoimmune haemolytic anTmia.1
remains
no bedsores, even at the terminal To confirm this impression all A.L.S. patients in our clinic were surveyed. From 1965 to 1975 202 patients were diagnosed A.L.S. in our outpatient clinic. 36 were admitted and none had bedsores. Inquiries of families of patients not admitted revealed that 16 had died at home without bedsores. Indeed we could find no A.L.S. patient with this complication, A.L.S. is fully described in neurological textbooks, but bed sores are not mentioned. However, a century ago Charcot, lecturing on A.L.S. at the Salpetriere Hospital in Paris,’ stated: "il n’y a aucune tendencela formation des eschares" (there is no tendency to the formation of bedsores). No attention seems to have been paid to this comment. In A.L.s. only motor systems are involved and no sensory impairment is found. This might be said to contribute to the absence of bedsores, but patients with parkinsonism often develop severe decubital ulcers despite preservation of normal cutaneous sensation. We think that a tendency not to develop bedsores is one of the characteristics of A.L.s. Although the cause of A.L.s. is unknown, absence of bedsores may be related to possible skin changes in this disorder.3 Department of Neurology, TETSUO FURUKAWA University of Tokyo, YASUO TOYOKURA Tokyo, Japan 113
A.L.s.
is similar.
33-year-old man was found to lack both serum and secreIgA during a screening of sera from blood-donors. He is one of a group of 23 people with selective IgA deficiency studied by Us.lU Hypertension was found during a medical examination for admission to military service, and blood-pressure values have ranged from 200/120 to 170/110 mm Hg. Polycystic disease of both kidneys was demonstrated by intravenous pyelography, and an increased plasma-renin-activity (4-59 ng ml-’ h-1) was detected in February, 1974. Catarrhal inflammation of the upper respiratory tract was a recurrent complaint in this patient. Serum-IgA was undetectable, even when the radioactive variant of single radial immunodiffusion" was used. The remaining immunoglobulin levels were: IgG 1990 and IgM 153 mg/dl, and IgD 33 and IgE 30 i.u./ml. Agglutinating antibodies against sera from sheep, cow, and goat and cow’s skim milk were revealed by inhibition of passive haemagglutination, the positivity titres ranging from 1/16 to 1/64. No IgA-bearing peripheral lymphocytes were shown by direct immunofluorescence on three occasions. The patient’s chromosome karyotype was normal. Four siblings and two children of this man all had normal serum-IgA levels. His mother had a horseshoe kidney malformation but normal serum-IgA. In a review of over a hundred cases of selective IgA deficiency, Ammann and Hong’2 never found a combination of absent serum-IgA, multicystic kidney disease, and hypertension. In view of the heterogeneity of disease associated with IgA deficiency, we suggest that a larger number of cases be stu-
A tory
chance association is excluded. FRANCO DAMMACCO Department of Internal Medicine, GIACOMO LUCIVERO 2nd Clinica Medica, Policlinico, LORENZO BONOMO 70124-Bari, Italy
died before
a
AMYOTROPHIC LATERAL SCLEROSIS AND BEDSORES
SIR,-Amyotrophic lateral sclerosis (A.L.S.) was first deby Charcot in the middle of the 19th century. It is a progressive disorder, and the patient is usually confined to bed within a few years of onset. Our impression has been that scribed
PRESENTATION OF RECTAL LEIOMYOMA
SIR,-Anal fissure is
a common cause of anal pain and is and routinely successfully treated by anal dilatation. 1 would like to report a case in which anal pain was caused by different lesion for which dilatation was wholly unsuitable. A 54-year-old man was seen in outpatients complaining of increasingly frequent bouts of sharp pain in the rectum over the previous year. The pain was associated with defecation but also occurred on sitting or lying down. There had been no change in bowel habit and he had passed no blood or slime per rectum. Rectal examination was painful and a posterior skin tag was observed. Despite the rather atypical history posterior anal fissure was diagnosed and he was admitted for sigmoidoscopy and anal stretch. At outpatient follow-up one month after admission, the pa tient was still complaining of pain, and on careful examination a 1-5 5 cm diameter lump could be felt about 1 cm posterior to the anus in the midline. Palpation of the tumour produced the pain of which the patient had been complaining. This was thought at that time to be a small infected haematoma following the anal stretch, and the patient was readmitted for the lesion to be drained. At operation a firm tumour was excised which was well encapsulated and not attached to surrounding structures. Histological examination showed a benign tumour with the whorled pattern charactistic of a leiomyoma. When seen six weeks after the second operation the patient was free of pain. Leiomyoma arising from the internal anal sphincter is rare, but since pain is a frequent presenting feature of rectal leiomyoma and since 50% of such lesions are mahgnant4 I feel II is important that the diagnosis is excluded by careful palpation of the anal margin, preferably with a finger in the rectum, before anal dilatation for fissure.
I thank Prof. D.
Johnston for permission to report this case.
Department of Surgery, Bristol Royal Infirmary, Bristol BS2 8HW
Horowitz, S., Hong, R. in Immunodeficiency in Man and Animals (edited by D. Bergsma); vol. xi, p. 123. New York, 1975. 9. Blajchman, M. A., Hobbs, J. R., Dacie, J. V. Lancet, 1963, ii, 340. 10. Dammacco, F., Tursi, A., Lucivero, G., Bonomo, L. Unpublished. 11. Rowe, D. S. Bull. Wld Hlth Org. 1969, 40, 613. 12. Ammann, A. J., Hong, R. in Immunologic Disorders in Infants and Children (edited by E. R. Stiehm and V. A. Fulginiti); p. 199. Philadelphia, 1973.
R. G. HUGHES
8.
1. 2.
Toyokura, Y. Neurol. Med., Tokyo, 1975, 2, 215.
Charcot, J. M. Leçon sur les maladies du système nerveux faites à la Salpêtrière; vol. II, p. 237. Paris, 1880. 3. Fullmer, H. M. in Motor Neuron Diseases (edited by F. H. Norris.,Jr and L. T. Kurland); p. 242. New York, 1968. 4. Golden, T., Stout, A. P. Surgery Gynec Obstet. 1941, 73, 784.
knowledge, West et al.’ were the first to report selective IgA deficiency in patients with diseases of central nervous system, including epilepsy. Since we reported selective IgA deficiency in a patient with postencephalitic epilepsy in 1968we have paid special attention to the effect of anticonvulsants, es-
PHENYTOIN IN BENIGN INSULINOMA
our
pecially phenytoin, on IgA, and we have seen twenty-two patients (aged 7-56 years) of whom eight had epilepsy. Six of the patients were on phenytoin alone or in combination with phenobarbitone, one was on carbamazepine alone, and one was being treated with trimetadione and phenobarbititone. In addition, we found primary acquired agammaglobutinsemia (one case) and type-i dysgammagtobulinaemia (one case) in patients with epilepsy receiving phenytoin; these patients lacked both serum and secretory IgA. Two of the patients with selective IgA deficiency and epilepsy had recurrent repiratory infections, and two had recurrent fevers. In the case of agammaglobulinxmia, pneumonia and acute otitis media
common, whereas the patient with had recurrent attacks of pustular dysgammaglobulinaemia lesions, usually limited to the hands. Immunohaematological abnormalities are common in patients with selective IgA deficiency and in patients on anticonvulsants. One patient with selective IgA deficiency and epilepsy had systemic lupus erythematosus.7 In selective IgA deficiency circulating IgA-bearing B lymphocytes are usually normal but we have seen a case with fewer than 1% of IgA-bearing B lymphocytes.’ Antinuclear antibodies and anti-IgA antibodies were positive in some patients with immunodeficiency diseases and epilepsy, and of thirty-five epileptics treated with phenytoin, three had antinuclear antibodies and seven had anti-IgA antibodies.8 Moreover, in one patient an abnormal serum-IgA (5 mg/dl) rose to 20 mg/dl when phenytoin was withdrawn, in parallel with a rise in the numbers of IgA-containing plasma-cells in the jejunal mucosa. This case suggests that IgA deficiency in patients receiving phenytoin is not always irreversible.9 Two other non-epileptic patients whose serum-IgA became undetectable had autoimmune diseases-namely, systemic lupus erythematosus and periarteritis nodosa.OJ We conclude that: (1) Phenytoin can mainly affect the IgA system, but sometimes other immunoglobulins also. 10 (2) Selective IgA deficiency may be induced11by the drug through the development of autoimmunity. Besides the defective secretion or increased catabolism of IgA, the suppression of lymphoid tissue by anti-IgA antibodies may be implicated in the pathogenesis of13selective IgA deficiency, as shown in animal experiments. 12 (3) Phenytoin may directly affect IgA-producing cells in the gut-associated lymphoid tissues. (4) Autoimmunity, induced by the drug could occur in conjunction with immunodeficiency diseases. Anticonvulsants, mainly phenytoin, 8 have been linked with a broader spectrum of immunopathies, including lymphoma. These findings would be pertinent to the pathogenesis of malignant lymphomas, which also can be induced by the drug."I were
First Division, Department of Internal Medicine,
Kyoto University, Kyoto 606, Japan.
T. KANOH H. UCHINO
5. West, C. D., Hong, R., Holland, N. H. J. clin. Invest. 1962, 41, 2054. 6. Kanoh, T., Wakisaka, G. Acta hâem. jap. 1968, 31, 481. 7. Takigawa, M., Kanoh, T., Imamura, S., Takahashi, C. Archs Derm. (in the press). 8 Kanoh, T., Yamaguchi, N. Excerpta med. int. Congr. Ser. 1973, no. 300, p. 71. 9. Kanoh, T., Niwa, Y., Yamaguchi, N. J. Jap. Soc. intern. Med. 1975, 64, 1353. 10 Grob, P. J., Herold, G. E. Br. med. J. 1972, ii, 561. 11. Kanoh, T. Acta hâem. jap. 1971, 34, 452. 12 Manning, D. D. J. Immun. 1972, 109, 1152. 13 Sorrell, T. C., Forbes, I. J., Burness, F. R., Rischbirth, R. H. C. Lancet,
1971, ii, 1233.
(diphenylhydantoin) inhibits insulin blood-levels similar to those effective in treating epilepsy.12 It inhibits insulin release from the labile and the large storage pools of insulin in the B cells. We have studied the effect of phenytoin on glucose and insulin concentrations in a 32-year-old man with symptoms of insulin excess. Laparotomy revealed a benign 2 x2 cm insulinoma in the head of the panSIR,-Phenytoin
release
at
creas.
Before operation, a prolonged fasting test was carried out and was repeated on the third day of oral phenytoin treatment (given in divided doses, 600 mg daily for two days and 300 mg on the third day). Serum-glucose and plasma-immunoreactiveinsulin (I.R.I.) were measured every 6 h: Time
Glucose
I.R.I.
(h offasting) Before phenytozn:
(mg/d/)
([LU/Ml)
12 18 24 28
74 50 12 22
8.0 6.0 6.0 6.0
Mean±S.E.M.
39-5±14.0
6.5:t0.5
60 60 29 40
4.0 4.0 4.0 3.0
15.0 15.0 7.25
47.3:t13.3
3.7:t0.25
12.6:t1.84
Glucose/I.R.I. 9.25 8.33 2.00 3.66
5.81:t1.75
After phenytoin: 12 18 24 28 Mean+S.E.M.
13.3
During phenytoin therapy, there was a significant rise in the
glucose/!.R.I. ratio although the change in glucose was not significant. Although there was a significant fall in plasma-i.R.I., the baseline was not high, suggesting that the tumour was secreting principally proinsulin.3 The effects of acute phenytoin treatment are similar to those previously reported .4 5 Future studies on the long-term effects of this treatment, especially in patients with unresectable tumours, would be of interest. Department of Internal Medicine, American University of Beirut Hospital, Beirut, Lebanon
MOH’D AMIN ARNAOUT IBRAHIM SALTI
SELECTIVE IgA DEFICIENCY AND IDIOPATHIC THROMBOCYTOPENIC PURPURA
SiR,—You have lately reviewed6
new
concepts in selective
IgA deficiency. The development in these patients of a variety of autoimmune disorders is established, but only one case of idiopathic thrombocytopenic purpura (I. ToP.) has been noted.7 We wish to report two such instances. Case 1.-A 19-year-old male was referred with a 1-year history of easy bruising. Slight splenomegaly was found, and I. T.P. was diagnosed. IgA was undetectable by radial immunodiffusion, the IgM level was normal; a polyclonal increase of IgG (2 g/dl) was noted. There was no pertinent past history.
Familial studies including immunoglobulin levels were unremarkable. Coombs test, antinuclear factors, and rheumatoid factors were negative. T-cell function was normal. After an unsuccessful course of steroids, the spleen was removed and the platelet-count returned to normal. Case 2.-A 40-year-old woman had had, for the past 5 years, mild sinusitis, tonsillitis, and recurrent urinary-tract infections. The findings of severe thrombocytopenia led to further evaluation. There was a slight splenomegaly and a mild Raynaud’s phenomenon. She had no IgA, with a poly1. Malherbe, C., Burril, K. C., Levin, S. R., Karam, J. H., Forsham, P. H. New
Engl. J. Med. 1972, 286, 339. 2.
Levin, S. R., Grodsky, G. M., Hagura, R., Smith, D. Diabetes, 1972, 21,
856. 3. Sherman, B. M., Pek, E., Fajans, S. S., Floyd, J. C., Conn, J. W. J. clin. Endocr. Metab. 1972, 35, 271. 4. Cotten, M. S., Bower, R. H., Fidler, S. M., Johnsonbaugh, R. E., Sode, J. Lancet, 1973, i, 40. 5. Pelkoner, R., Taskinen, M. R. ibid. p. 604. 6. Lancet, 1975, ii, 1291. 7. Hong, R., Ammann, A. J. Am. J. Path. 1972, 69, 491.
862
IgM and IgG (2-5 g/dl); a circulating anticephalin anticoagulant; and positive Coombs test with elution of an IgG anti-e antibody without overt haemolysis during a 2-year period. It is thus probable that I.T.P. may be associated with IgA deficiency although the true incidence of this disorder in I.T.P.
clonal increase of
,
to
Laboratory of Immunochemistry (INSERM Research Institute on Blood
Diseases,
Hôpital Saint-Louis, 75475 Paris Cedex 10, France
U
108)
JEAN-CLAUDE BROUET MAXIME SELIGMANN
POLYCYSTIC RENAL DISEASE, HYPERTENSION, AND IgA DEFICIENCY
SIR,-Dr Gabriel (Jan. 24, p. 197) raises the possibility that hypertension, multicystic disease of the kidney, and IgA deficiency might not be a fortuitous combination. The following case
patients develop
stages.’
be established. Since a subtle T-cell defect has been postulated in IgA deficiency it is advisable to evaluate carefully T-cell function in such patients when splenectomy is recommended. Low levels of IgA or selective IgA deficiency have been previously found in patients with autoimmune haemolytic anTmia.1
remains
no bedsores, even at the terminal To confirm this impression all A.L.S. patients in our clinic were surveyed. From 1965 to 1975 202 patients were diagnosed A.L.S. in our outpatient clinic. 36 were admitted and none had bedsores. Inquiries of families of patients not admitted revealed that 16 had died at home without bedsores. Indeed we could find no A.L.S. patient with this complication, A.L.S. is fully described in neurological textbooks, but bed sores are not mentioned. However, a century ago Charcot, lecturing on A.L.S. at the Salpetriere Hospital in Paris,’ stated: "il n’y a aucune tendencela formation des eschares" (there is no tendency to the formation of bedsores). No attention seems to have been paid to this comment. In A.L.s. only motor systems are involved and no sensory impairment is found. This might be said to contribute to the absence of bedsores, but patients with parkinsonism often develop severe decubital ulcers despite preservation of normal cutaneous sensation. We think that a tendency not to develop bedsores is one of the characteristics of A.L.s. Although the cause of A.L.s. is unknown, absence of bedsores may be related to possible skin changes in this disorder.3 Department of Neurology, TETSUO FURUKAWA University of Tokyo, YASUO TOYOKURA Tokyo, Japan 113
A.L.s.
is similar.
33-year-old man was found to lack both serum and secreIgA during a screening of sera from blood-donors. He is one of a group of 23 people with selective IgA deficiency studied by Us.lU Hypertension was found during a medical examination for admission to military service, and blood-pressure values have ranged from 200/120 to 170/110 mm Hg. Polycystic disease of both kidneys was demonstrated by intravenous pyelography, and an increased plasma-renin-activity (4-59 ng ml-’ h-1) was detected in February, 1974. Catarrhal inflammation of the upper respiratory tract was a recurrent complaint in this patient. Serum-IgA was undetectable, even when the radioactive variant of single radial immunodiffusion" was used. The remaining immunoglobulin levels were: IgG 1990 and IgM 153 mg/dl, and IgD 33 and IgE 30 i.u./ml. Agglutinating antibodies against sera from sheep, cow, and goat and cow’s skim milk were revealed by inhibition of passive haemagglutination, the positivity titres ranging from 1/16 to 1/64. No IgA-bearing peripheral lymphocytes were shown by direct immunofluorescence on three occasions. The patient’s chromosome karyotype was normal. Four siblings and two children of this man all had normal serum-IgA levels. His mother had a horseshoe kidney malformation but normal serum-IgA. In a review of over a hundred cases of selective IgA deficiency, Ammann and Hong’2 never found a combination of absent serum-IgA, multicystic kidney disease, and hypertension. In view of the heterogeneity of disease associated with IgA deficiency, we suggest that a larger number of cases be stu-
A tory
chance association is excluded. FRANCO DAMMACCO Department of Internal Medicine, GIACOMO LUCIVERO 2nd Clinica Medica, Policlinico, LORENZO BONOMO 70124-Bari, Italy
died before
a
AMYOTROPHIC LATERAL SCLEROSIS AND BEDSORES
SIR,-Amyotrophic lateral sclerosis (A.L.S.) was first deby Charcot in the middle of the 19th century. It is a progressive disorder, and the patient is usually confined to bed within a few years of onset. Our impression has been that scribed
PRESENTATION OF RECTAL LEIOMYOMA
SIR,-Anal fissure is
a common cause of anal pain and is and routinely successfully treated by anal dilatation. 1 would like to report a case in which anal pain was caused by different lesion for which dilatation was wholly unsuitable. A 54-year-old man was seen in outpatients complaining of increasingly frequent bouts of sharp pain in the rectum over the previous year. The pain was associated with defecation but also occurred on sitting or lying down. There had been no change in bowel habit and he had passed no blood or slime per rectum. Rectal examination was painful and a posterior skin tag was observed. Despite the rather atypical history posterior anal fissure was diagnosed and he was admitted for sigmoidoscopy and anal stretch. At outpatient follow-up one month after admission, the pa tient was still complaining of pain, and on careful examination a 1-5 5 cm diameter lump could be felt about 1 cm posterior to the anus in the midline. Palpation of the tumour produced the pain of which the patient had been complaining. This was thought at that time to be a small infected haematoma following the anal stretch, and the patient was readmitted for the lesion to be drained. At operation a firm tumour was excised which was well encapsulated and not attached to surrounding structures. Histological examination showed a benign tumour with the whorled pattern charactistic of a leiomyoma. When seen six weeks after the second operation the patient was free of pain. Leiomyoma arising from the internal anal sphincter is rare, but since pain is a frequent presenting feature of rectal leiomyoma and since 50% of such lesions are mahgnant4 I feel II is important that the diagnosis is excluded by careful palpation of the anal margin, preferably with a finger in the rectum, before anal dilatation for fissure.
I thank Prof. D.
Johnston for permission to report this case.
Department of Surgery, Bristol Royal Infirmary, Bristol BS2 8HW
Horowitz, S., Hong, R. in Immunodeficiency in Man and Animals (edited by D. Bergsma); vol. xi, p. 123. New York, 1975. 9. Blajchman, M. A., Hobbs, J. R., Dacie, J. V. Lancet, 1963, ii, 340. 10. Dammacco, F., Tursi, A., Lucivero, G., Bonomo, L. Unpublished. 11. Rowe, D. S. Bull. Wld Hlth Org. 1969, 40, 613. 12. Ammann, A. J., Hong, R. in Immunologic Disorders in Infants and Children (edited by E. R. Stiehm and V. A. Fulginiti); p. 199. Philadelphia, 1973.
R. G. HUGHES
8.
1. 2.
Toyokura, Y. Neurol. Med., Tokyo, 1975, 2, 215.
Charcot, J. M. Leçon sur les maladies du système nerveux faites à la Salpêtrière; vol. II, p. 237. Paris, 1880. 3. Fullmer, H. M. in Motor Neuron Diseases (edited by F. H. Norris.,Jr and L. T. Kurland); p. 242. New York, 1968. 4. Golden, T., Stout, A. P. Surgery Gynec Obstet. 1941, 73, 784.