Volume 88 Number 2
Letters to the Editor
Behavior of the complement system in the nephropathy of congenital syphilis To the Editor: The incidence of nephropathy as a complication of congenital syphilis is difficult to assess, but it does seem to be a rather uncommon complication in syphilitic infection. 1 The precise pathogenic mechanisms are not known; recent studies suggest that the nephropathy might be related to the deposition of immune complexes on the epithelial surface of the basement membrane of the renal glomeruli. Several workers have demonstrated immunoglobulin deposition with or without C3 in tissue from kidney biopsies of infants with congenital syphilis and in adults with secondary syphilis. 2-~ Serum concentration of different complement components have been extensively studied by several investigators in different types of nephritis, '-~ but not in the nephropathy of congenital syphilis. We have assumed that these determinations may provide useful information regarding the pathogenesis of this disorder. Measurements of the serum levels of the complement components Clq, C4, C3, and C5 were performed in two patients with congenital syphilis: one with the nephrotic syndrome and the other with acute glomerulonephritis. Quantitations were performed by radial immunodiffusion with specific antisera donated by courtesy of Dr. H. J. Miiller Eberhard (U.S.A.). Each of the infants had typical clinical features of congenital syphilis. One infant (Case 1), a 4-month-old female, had clinical and laboratory evidence of the nephrotic syndrome, and the other one (Case 2), a 50-day-old male infant had urinary findings compatible with the diagnosis of glomerulonephritis. Each infant had a positive VDRL test and responded satisfactorily to treatment with penicillin. Table I shows the data from our patients and the normal values (mean _+ 1 SD) obtained in 20 normal infants 1 to 4 months of age. The finding of low levels of the earlier components of the complement system in each patient suggests classical pathway activation of the complement cascade. This complement profile is similar to that of the nephritis of systemic lupus erythematosus, in Table I. S e r u m levels of CH50, Clq, C4, C3, C5, a n d C3PA in two infants with congenital syphilis
CH50 Clq C4 (C;/ (U/ (mg/ (mg/ ml) dl) dl) dl) Normal values (mean _+ 1 SD) Case 1 Case 2* Case 2] *Before treatment. tAller treatment.
(C;/ C3PA (% of dl) N)
243 14 43.7 122 7.2 104 _+32 _+2.5 +9.6 +23 _+2.2 _+ 11 0 6.6 19.2 57.6 8.9 -0 2.2 4.5 51.2 7.8 I00 105 18 24 152 10 148
359
which the complement system is activated by circulating immune complexes, although recent studies have demonstrated that the C3b feedback mechanism may also play a rote, activating the C3PA system.6 In Case 2 the normal values of C3PA are difficult to evaluate; therefore one cannot say that the bypass mechanism is not functioning in this type of nephritis. A follow-up study in Case 2 showed that with clinical improvement the values of the complement components returned to normal, with the exception of C4, that persisted low as it frequently does in lupus nephritis. The presented data support the immunopathologic findings of several workers in the sense that the nephropathy of congenital syphilis may be due to the deposition of circulating immune complexes.
M. Zelazko G. Feldman Sector de Inmunologia (Departmento de Medicina) y Centro de Estudios Pediiltrieos Hospital de Nihos. Gallo 1330 Buenos A ires. Argentina REFERENCES
1. Saxoni F, Lapatsanis P, and Pantelakis SN: Congenital syphilis: A description of 18 cases and re-examination of an old but ever-present disease, Clin Pediatr 6:687, 1967. 2. Braunstein GD, Lewis E J, Galvanek EG, Hamilton A, and Bell WR: The nephrotic syndrome associated with secondary syphilis. An immune deposit disease, Am J Med 48:643, 1970. 3. Kaplan B, Wiglesworth W, Marks M, and Drummond K: The glomerulopathy of congenital syphilis--an immune deposit disease, J PEDIATR 81:1154, 1972. 4. Kohler PF, and Ten Bensel R: Serial complement component alterations in acute glomerulonephritis and systemic lupus erythematosus, Clin Exp Immunol 4:191, 1969. 5. Cameron JS, Vick RM, Ogg C, Seymour WM, Chantler C, and Turner DR: Plasma C3 and C4 concentrations in management of glomerulonephritis, Br Med J 3:668, 1973. 6. McLean R, and Michael AF: Properdin and C3 proactivator. Alternate pathway components in human glomerulonephritis, J Clin Invest 52:634, 1973.
A possible relationship of nevus sebaceous of Jadassohn (organoid nevus) to childhood malignancies To the Editor: The association of congenital anomalies with certain childhood malignancies has been described? -~ The purpose of this letter is to report the occurrence of nevus sebaceous of Jadassohn in two of 40 consecutive children with acute lymphocytic leukemia and in one of six children with rhabdomyosarcoma. These three
Letters to the Editor
Behavior of the complement system in the nephropathy of congenital syphilis To the Editor: The incidence of nephropathy as a complication of congenital syphilis is difficult to assess, but it does seem to be a rather uncommon complication in syphilitic infection. 1 The precise pathogenic mechanisms are not known; recent studies suggest that the nephropathy might be related to the deposition of immune complexes on the epithelial surface of the basement membrane of the renal glomeruli. Several workers have demonstrated immunoglobulin deposition with or without C3 in tissue from kidney biopsies of infants with congenital syphilis and in adults with secondary syphilis. 2-~ Serum concentration of different complement components have been extensively studied by several investigators in different types of nephritis, '-~ but not in the nephropathy of congenital syphilis. We have assumed that these determinations may provide useful information regarding the pathogenesis of this disorder. Measurements of the serum levels of the complement components Clq, C4, C3, and C5 were performed in two patients with congenital syphilis: one with the nephrotic syndrome and the other with acute glomerulonephritis. Quantitations were performed by radial immunodiffusion with specific antisera donated by courtesy of Dr. H. J. Miiller Eberhard (U.S.A.). Each of the infants had typical clinical features of congenital syphilis. One infant (Case 1), a 4-month-old female, had clinical and laboratory evidence of the nephrotic syndrome, and the other one (Case 2), a 50-day-old male infant had urinary findings compatible with the diagnosis of glomerulonephritis. Each infant had a positive VDRL test and responded satisfactorily to treatment with penicillin. Table I shows the data from our patients and the normal values (mean _+ 1 SD) obtained in 20 normal infants 1 to 4 months of age. The finding of low levels of the earlier components of the complement system in each patient suggests classical pathway activation of the complement cascade. This complement profile is similar to that of the nephritis of systemic lupus erythematosus, in Table I. S e r u m levels of CH50, Clq, C4, C3, C5, a n d C3PA in two infants with congenital syphilis
CH50 Clq C4 (C;/ (U/ (mg/ (mg/ ml) dl) dl) dl) Normal values (mean _+ 1 SD) Case 1 Case 2* Case 2] *Before treatment. tAller treatment.
(C;/ C3PA (% of dl) N)
243 14 43.7 122 7.2 104 _+32 _+2.5 +9.6 +23 _+2.2 _+ 11 0 6.6 19.2 57.6 8.9 -0 2.2 4.5 51.2 7.8 I00 105 18 24 152 10 148
359
which the complement system is activated by circulating immune complexes, although recent studies have demonstrated that the C3b feedback mechanism may also play a rote, activating the C3PA system.6 In Case 2 the normal values of C3PA are difficult to evaluate; therefore one cannot say that the bypass mechanism is not functioning in this type of nephritis. A follow-up study in Case 2 showed that with clinical improvement the values of the complement components returned to normal, with the exception of C4, that persisted low as it frequently does in lupus nephritis. The presented data support the immunopathologic findings of several workers in the sense that the nephropathy of congenital syphilis may be due to the deposition of circulating immune complexes.
M. Zelazko G. Feldman Sector de Inmunologia (Departmento de Medicina) y Centro de Estudios Pediiltrieos Hospital de Nihos. Gallo 1330 Buenos A ires. Argentina REFERENCES
1. Saxoni F, Lapatsanis P, and Pantelakis SN: Congenital syphilis: A description of 18 cases and re-examination of an old but ever-present disease, Clin Pediatr 6:687, 1967. 2. Braunstein GD, Lewis E J, Galvanek EG, Hamilton A, and Bell WR: The nephrotic syndrome associated with secondary syphilis. An immune deposit disease, Am J Med 48:643, 1970. 3. Kaplan B, Wiglesworth W, Marks M, and Drummond K: The glomerulopathy of congenital syphilis--an immune deposit disease, J PEDIATR 81:1154, 1972. 4. Kohler PF, and Ten Bensel R: Serial complement component alterations in acute glomerulonephritis and systemic lupus erythematosus, Clin Exp Immunol 4:191, 1969. 5. Cameron JS, Vick RM, Ogg C, Seymour WM, Chantler C, and Turner DR: Plasma C3 and C4 concentrations in management of glomerulonephritis, Br Med J 3:668, 1973. 6. McLean R, and Michael AF: Properdin and C3 proactivator. Alternate pathway components in human glomerulonephritis, J Clin Invest 52:634, 1973.
A possible relationship of nevus sebaceous of Jadassohn (organoid nevus) to childhood malignancies To the Editor: The association of congenital anomalies with certain childhood malignancies has been described? -~ The purpose of this letter is to report the occurrence of nevus sebaceous of Jadassohn in two of 40 consecutive children with acute lymphocytic leukemia and in one of six children with rhabdomyosarcoma. These three
