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Congenital complete heart block associated with maternal mixed connective tissue disease
420
B r i e f clinical and laboratory observations
adrenal tumour in the mother, Acta Endocrinol 65:627, 1970. 7 . Harrison KA, and Lister UG: Successful pregnancy in a patient with adrenogenital syndrome, J Obstet Gynaecol Br Comm 73:493, 1966: 8. Riddick DH, and Hammond CB: Adrenal virilism due to 21-hydroxylase deficiency in the postmenarchial female, Obstet Gynecol 45:21, 1975. 9. Leichter SB, and Jacobs LS: Normal gestation and dimin-
The Journal of Pediatrics September 1979
ished androgen responsiveness in an untreated patient with 21-hydroxylase deficiency, J Clin Endocrinol Metab 42:575, 1976. 10. Klingensmith GJ, Garcia SC, Jones HW, and Migeon CJ: Linear growth, age of menarche, and pregnancy rates in females with steroid-treated cor/genital adrenal hyperplasia at the Johns Hopkins Hospital, in Lee PA, Plotnick LP, Kowarski AA, and Migeon CJ, editors: Congenital adrenal hyperplasia, Baltimore, 1977, University Park Press.
Congenital complete heart block associated with maternal mixed connective tissue disease Robert J. Nolan, B.S., Stanford T. Shulman, M.D., and Benjamin E. Victoriea, M.D.,*Gainesville, Fla.
THE R E L A T I O N S H I P b e t w e e n congenital complete heart block a n d m a t e r n a l collagen-vascular disease was established more t h a n a d e c a d e ago by Hull et al. 1 R e c e n t reviews h a v e r e - e m p h a s i z e d that m a t e r n a l collagenvascular disease, s y m p t o m a t i c or asymptomatic, m a y b e a cause of C C H B . C C H B has b e e n r e p o r t e d in the offspring of w o m e n with systemic l u p u s e r y t h e m a t o s u s or with r h e u m a t o i d arthritis. ~' 3 W e report a n i n f a n t with C C H B , who s u b s e q u e n t l y d e v e l o p e d pericardial t a m p o n a d e a n d a discoid lupus rash. T h e p a t i e n t ' s m o t h e r was f o u n d to have e v i d e n c e o f mixed connective tissue disease. T h e association of m a t e r n a l M C T D a n d C C H B has not b e e n reported previously. CASE REPORT Patient S. W., a black female, was the 1,540 gm product of a 32-week gestation to a 30-year-old para 3-0-0-3. Persistent bradycardia of 55/minute was noted at birth and an electrocardiogram revealed congenital complete heart block with an atrial rate of 1407"minute and a ventricular rate of 55/minute. The infant was transported to the Shands Teaching Hospital (University of Florida) for further evaluation9 On admission she had mild tachypnea, normal heart sounds, and no significant murmurs 9 The skin was clear. Chest radiograph demonstrated moderate cardiomegaly and normal lung fields. A hematocrit was 48% and serum electrolyte values were normal9 Rheumatoid factor and anti-deoxyribonucleoprotein values were negative, but a speckled pattern anti-nuclear antibody was positive at a titer of 1:640 (Table). A cytomegalovirus titer (complement fixation) was positive at 1:256. She remained clinically stable with CCHB and was discharged at one month of age. Repeat CMV titer at that time was 1:256. At two months of age she developed a severe scaling erythematous rash on her face and upper extremities. At 4 months of age she was readmitted w i t h cardiac tamponade. Multiple well*Reprint address: Department of Pediatrics, Division of Pediatric Cardiology, University of Florida College of Medicine, Box J-296, Gainesville, FL 32610.
demarcated atrophic macules with overlying telangiectasia were noted, especially on the face, head, and upper torso9 The upper eyelids were noted to be violeceous (Figure). She had tachypnea and coarse rhonchi, rales, and end-expiratory wheezes bilaterally. Heart sounds were distant. The liver was firm and palpable 6 cm below the right costal margin. Abbreviations used CCHB: congenital complete heart block MCTD: mixed connective tissue disease CMV: cytomegalovirus CPK: creatinine phosphokinase SLE: systemic lupus erythematous A large cardiac silhouette was present on admission chest radiograph. Echocardiogram confirmed the presence of a significant pericardial effusion. Sixty milliliters of sterile amber fluid containing 84 RBC/mm ", 7 WBC/mm 8, glucose 104 mg/dl, and protein 4.6 gm/dl were removed by pericardiocentesis. After this procedure the infant improved markedly and had normal heart sounds, and a sharp soft liver edge 3 cm below the costal margin9 Because pericardial fluid reaccumulated during the next week, a pericardial window was created. An involved area of slain obtained by biopsy was positive by immunofluorescence for C3 and fibrinogen at the dermalepidermal junction, findings consistent with a discoid lupus rash9 Laboratory evaluation included normal hematologic studies, serum electrolyte concentrations, total protein, albumin, liver function tests, VDRL, and hepatitis B surface antigen9 Total CPK was mild!y elevated at 190 m l U / m l (upper limit of normal at four months 99 mlU/ml). Immunologic studies were repeated (Table). Urinalysis was negative. Repeated CMV titers demonstrated no antibody. There were no titers to other significant viruses (e.g., Coxsackie). The infant has remained clinically stable. Repeat echocardiogram showed no residual pericardial effusion, and normal left ventricular size and function. By 7 months of age the skin lesions showed nearly complete resolution. Patient S. W.'s mother had a two-year history of the Raynaud phenomenon, and mild dysphagia with associated heartburn 9 002273476/79/090420+03500.30/0 9 1979 The C. V. MosbyCo.
Volume 95 Number 3
Brief clinical and laboratory observations
4 21
Figure. Patient S. W. at 4 months of age displaying a characteristic discoid lupus rash. Despite her minor symptoms, she had striking laboratory abnormalities: total protein 11.7 gm/dl, albumin 3.8 gm/dl, and CPK 1960 mlU/1 (upper normal- 180 mIU/1). CMV titer was positive at1:256. Extensive immunologic studies were obtained (Table). Other laboratory findings included slightly elevated LDH and SGOT, normal total and direct bilirubin, hematocrit, urinalysis, negative VDRL, rheumatoid factor, and hepatitis B surface antigen. DISCUSSION Mixed connective tissue disease is a distinct, recently described c~ syndrome combining manifestations of systemic lupus erythematosus, polymyositis, progressive systemic sclerosis, and rheumatoid arthritis.'. ~ The findings of the R a y n a u d p h e n o m e n o n , disordered esophageal motility, asymptomatically elevated CPK levels, hypergammaglobulinemia, and classic serology in our patient's mother are diagnostic of MCTD. M C T D is characterized by high titers of speckled pattern antinuclear antibody, and the invariable presence of very high antibody titers (from 1:1000 to greater than 1:1,000,000) to the ribonucleoprotein antigen fraction of extractable nuclear antigen, ~. 6 The low maternal titer to t h e Sm antigen fraction of extractable nuclear antigen, occasionally described in MCTD, and her normal D N A binding~ rarely seen in active untreated SLE, are consistent with maternal MCTD. '-~ Complete heart block as a result of focal degeneration and fibrosis of the conduction system is well described in adults with SLE, progressive systemic sclerosis, rheumatoid arthritis, polyarteritis n o d o s a , dermatomyositis, and polymyositis.~. 7.8 The pathologic changes noted in the conduction systems of neonates with CCHB born to mothers with SLE resemble those seen in adults with SLE and complete heart block? Our patient's skin lesions were
Table. Selected immunologic data Patient
Age Anti-DNP* Anti-DNA* Speekled pattern ANA* Anti-DNAt Anti-SM:~ Anti-RNP~ IgGw IgMw IgAw
Mother
1 day Neg Neg
4 mo Neg Neg
5 mo Neg Neg
1:640 920 19 < 0.45
1:80 1% Neg 1:80 500 161 207
1:20 ---
Neg Neg 1:2560 1% 1:640 1:1,000,000 5,010 155 136
Abbreviations used: Anti-DNP= anti-deoxyribonucleoprotein; antiDNA = anti-deoxyribonucleic acid; ANA = anti-nuclear antibody; SM = SM antigen fraction (RNase-insensitivefraction) of extractable nuclear antigen; RNP = ribonucleoprotein fraction (RNase-sensitive fraction) of extractable nuclear antigen. *lmmunofluorescence. t% Binding to native DNA by radioimrnunoassay(normal < 20%). :~ScrippsReference Laboratory, La Jolla, Calif. w immunodiffusion(mg/dl). similar to those of neonatal discoid and systemic lupus described by Vonderheid et a P - i n i t i a l l y scaling erythematous macules resolving with time t O hypopigmented, atrophic, telangiectatic lesions usually confined to the upper body. Although the neonatal skin lesions associated with maternal collagen-vascular disease usually resolve completely, 9 the CCHB associated with maternal collagen-vascular disease has been p e r m a n e n t in almost all reported cases. The unusual concomitant occurrence of pericarditis and CCHB in our patient, unreported in Michaelsson and Engle's comprehensive study of 599 children with CCHB, 19 suggests a common immunopathologic basis for both lesions. Six of 14 children with M C T D in Singsen's
422
The Journal of Pediatrics September 1979
Brief clinical and laboratory observations
pediatric series developed pericarditis, indicating the relatively high frequency of this complication in the pediatric age group? The transplacental passage of IgG antinuclear antibo dies suggests their etiologic role in the development of CCHB~ pericarditis, and the skin lesions of neonatal lupus in our patient. ~. 11 The decay ofth e speckled pattern A N A titer in our patient, with a half-life of approximately 30 days, parallels the established decay curves for passively acquired maternal IgG. Congenital CMV infection was initially considered in the differential diagnosis of our patient because of positive CMV titers and a serum IgM of 19 mg/dl on the first day of life. Follow-up CMV titers demonstrated the disappearance of these antibodies. The finding of a CMV titer of 1:256 in the mother corroborates a recent report demonstrating persistently elevated CMV titers in patients with MCTDY The significance of this observa"tion is unclear but may reflect the generalized hypergammaglobulinemia present in these patients. It is increasingly apparent that CCHB may result from a Wide variety of maternal collagen-vascular disorders including MCTD: clinicians should be alert to the as yet undescribed possibility of conduction disorders in the older child or adult with MCTD. REFERENCES
1. Hull D. Binns BAO. and Joyce D: Congenital heart block and Widesp!"eadfibrosis due to maternal lupus erythematosus, Arch Dis Child 41:688, 1966.
2. McCue CM, Mantakas ME, Tingelstad JB, and Ruddy S: Congenital heart block in newborns of mothers with connective tissue disease, Circulation 56:82, 1977. 3. Chameides L, Truex RC, Vetter V, et al.: Association of maternal systemic lupus eryt.hematosus with congenital complete heart block, N Engl J Med 297:1204, 1977. 4. Sharp GC, Irvin wS, May CM, et al.: Association of antibodies to ribonucleoprotein and Sm antigens wish mixed connective tissue disease, systemic lupus erythematosus and other rheumatic diseases, N Engl J Med 295:1149, 1976. 5. Singsen BH, Bernstein BH, Kornreich HK, Koster King K, Hanson V, and Tan EM: Mixed connective tissue disease in childhood, J PEDIATR90:893, 1977. 6. Notman DD, Kurata N, Tan EM: profiles of antinuclear antibodies in systemic rheumatic diseases, Ann Intern Med 83:464, 1975. 7. Bharati S, de la Fuente DJ, Kallen RJ, et al.: Conduction system in systemic lupus erythematosus with atrioventricu lar block, Am J Cardiol 35:299, 1975. 8. Schauburg HH, Nielsen SL, Yurchak PM: Heart block in polymyositis, N Engl J Med 284:480, 19'71. 9. Vonderheid EC, Koblenzer PJ, Ming PML, and Burgoon CF: Neonatal lupus erythematosus, Arch Dermatol 112:698, 1976. 10. Michaelsson M, and Engle MA: Congenital complete heart block: An international study of the natural history, Cardiovasc Clin 4:84, 1972. 11. Beck JS, Oakley CL, and Rowell NR: Transplacental passage of antinuciear antibody, Arch Dermatol 93:656, 1966. 12. Rosenthal M: Cytomegaly virus antibodies in MCTD, Arthritis Rheum 20:762, 1977.
The relationship of diapers to diaper rashes in the one-month-old infant Fred Wiener, M.D., C.M., F.R.C.P.(C), Montreal, P.Q., Canada
THE CONTRIBUTION of the diaper to rashes in the diaper area has b e e n a point Of contention for many years. The layette for babies in the early decades consisted of various cloths and Y~soakers." The last was a wool overgarment simiIar in style and function to present-day plastic pants? In the 1930s and 40s, rubber pants became popular but were available in larg e sizes only. The smaller baby wore diapers with or without woven or knitted pants, and was usually positioned on a quilted pad. All of the above techniques drew urine away from the baby's diaper area and allowed air to enter. Repri m address." 6212 Clanranald A re, Montreal, P. Q., Canada H 3 X 2T3.
During the 1950s, plastic pants in sizes suitable for infants under three months became popular. It is probably more than coincidence that monilial diaper ras h was recognized as an important entity at that time. ~ Burgoon and Wright ~ listed several predisposing and activating factors, including ',maceration caused by continuous contact ,with a wet diaper and increased by the moist heat produce d by an impervious rubber cover." Thus, the technologic advance of occluding the small baby's bottom with impervious rubber pants may have led to the considerable frequency of the monilial variety of diaper rash. The convenience and watertight properties of the disposable plasticized paper diape r led to its widespread
0022=3476/79/090422 +03500.30/0 9 1979 The C. V. Mosby Co.
B r i e f clinical and laboratory observations
adrenal tumour in the mother, Acta Endocrinol 65:627, 1970. 7 . Harrison KA, and Lister UG: Successful pregnancy in a patient with adrenogenital syndrome, J Obstet Gynaecol Br Comm 73:493, 1966: 8. Riddick DH, and Hammond CB: Adrenal virilism due to 21-hydroxylase deficiency in the postmenarchial female, Obstet Gynecol 45:21, 1975. 9. Leichter SB, and Jacobs LS: Normal gestation and dimin-
The Journal of Pediatrics September 1979
ished androgen responsiveness in an untreated patient with 21-hydroxylase deficiency, J Clin Endocrinol Metab 42:575, 1976. 10. Klingensmith GJ, Garcia SC, Jones HW, and Migeon CJ: Linear growth, age of menarche, and pregnancy rates in females with steroid-treated cor/genital adrenal hyperplasia at the Johns Hopkins Hospital, in Lee PA, Plotnick LP, Kowarski AA, and Migeon CJ, editors: Congenital adrenal hyperplasia, Baltimore, 1977, University Park Press.
Congenital complete heart block associated with maternal mixed connective tissue disease Robert J. Nolan, B.S., Stanford T. Shulman, M.D., and Benjamin E. Victoriea, M.D.,*Gainesville, Fla.
THE R E L A T I O N S H I P b e t w e e n congenital complete heart block a n d m a t e r n a l collagen-vascular disease was established more t h a n a d e c a d e ago by Hull et al. 1 R e c e n t reviews h a v e r e - e m p h a s i z e d that m a t e r n a l collagenvascular disease, s y m p t o m a t i c or asymptomatic, m a y b e a cause of C C H B . C C H B has b e e n r e p o r t e d in the offspring of w o m e n with systemic l u p u s e r y t h e m a t o s u s or with r h e u m a t o i d arthritis. ~' 3 W e report a n i n f a n t with C C H B , who s u b s e q u e n t l y d e v e l o p e d pericardial t a m p o n a d e a n d a discoid lupus rash. T h e p a t i e n t ' s m o t h e r was f o u n d to have e v i d e n c e o f mixed connective tissue disease. T h e association of m a t e r n a l M C T D a n d C C H B has not b e e n reported previously. CASE REPORT Patient S. W., a black female, was the 1,540 gm product of a 32-week gestation to a 30-year-old para 3-0-0-3. Persistent bradycardia of 55/minute was noted at birth and an electrocardiogram revealed congenital complete heart block with an atrial rate of 1407"minute and a ventricular rate of 55/minute. The infant was transported to the Shands Teaching Hospital (University of Florida) for further evaluation9 On admission she had mild tachypnea, normal heart sounds, and no significant murmurs 9 The skin was clear. Chest radiograph demonstrated moderate cardiomegaly and normal lung fields. A hematocrit was 48% and serum electrolyte values were normal9 Rheumatoid factor and anti-deoxyribonucleoprotein values were negative, but a speckled pattern anti-nuclear antibody was positive at a titer of 1:640 (Table). A cytomegalovirus titer (complement fixation) was positive at 1:256. She remained clinically stable with CCHB and was discharged at one month of age. Repeat CMV titer at that time was 1:256. At two months of age she developed a severe scaling erythematous rash on her face and upper extremities. At 4 months of age she was readmitted w i t h cardiac tamponade. Multiple well*Reprint address: Department of Pediatrics, Division of Pediatric Cardiology, University of Florida College of Medicine, Box J-296, Gainesville, FL 32610.
demarcated atrophic macules with overlying telangiectasia were noted, especially on the face, head, and upper torso9 The upper eyelids were noted to be violeceous (Figure). She had tachypnea and coarse rhonchi, rales, and end-expiratory wheezes bilaterally. Heart sounds were distant. The liver was firm and palpable 6 cm below the right costal margin. Abbreviations used CCHB: congenital complete heart block MCTD: mixed connective tissue disease CMV: cytomegalovirus CPK: creatinine phosphokinase SLE: systemic lupus erythematous A large cardiac silhouette was present on admission chest radiograph. Echocardiogram confirmed the presence of a significant pericardial effusion. Sixty milliliters of sterile amber fluid containing 84 RBC/mm ", 7 WBC/mm 8, glucose 104 mg/dl, and protein 4.6 gm/dl were removed by pericardiocentesis. After this procedure the infant improved markedly and had normal heart sounds, and a sharp soft liver edge 3 cm below the costal margin9 Because pericardial fluid reaccumulated during the next week, a pericardial window was created. An involved area of slain obtained by biopsy was positive by immunofluorescence for C3 and fibrinogen at the dermalepidermal junction, findings consistent with a discoid lupus rash9 Laboratory evaluation included normal hematologic studies, serum electrolyte concentrations, total protein, albumin, liver function tests, VDRL, and hepatitis B surface antigen9 Total CPK was mild!y elevated at 190 m l U / m l (upper limit of normal at four months 99 mlU/ml). Immunologic studies were repeated (Table). Urinalysis was negative. Repeated CMV titers demonstrated no antibody. There were no titers to other significant viruses (e.g., Coxsackie). The infant has remained clinically stable. Repeat echocardiogram showed no residual pericardial effusion, and normal left ventricular size and function. By 7 months of age the skin lesions showed nearly complete resolution. Patient S. W.'s mother had a two-year history of the Raynaud phenomenon, and mild dysphagia with associated heartburn 9 002273476/79/090420+03500.30/0 9 1979 The C. V. MosbyCo.
Volume 95 Number 3
Brief clinical and laboratory observations
4 21
Figure. Patient S. W. at 4 months of age displaying a characteristic discoid lupus rash. Despite her minor symptoms, she had striking laboratory abnormalities: total protein 11.7 gm/dl, albumin 3.8 gm/dl, and CPK 1960 mlU/1 (upper normal- 180 mIU/1). CMV titer was positive at1:256. Extensive immunologic studies were obtained (Table). Other laboratory findings included slightly elevated LDH and SGOT, normal total and direct bilirubin, hematocrit, urinalysis, negative VDRL, rheumatoid factor, and hepatitis B surface antigen. DISCUSSION Mixed connective tissue disease is a distinct, recently described c~ syndrome combining manifestations of systemic lupus erythematosus, polymyositis, progressive systemic sclerosis, and rheumatoid arthritis.'. ~ The findings of the R a y n a u d p h e n o m e n o n , disordered esophageal motility, asymptomatically elevated CPK levels, hypergammaglobulinemia, and classic serology in our patient's mother are diagnostic of MCTD. M C T D is characterized by high titers of speckled pattern antinuclear antibody, and the invariable presence of very high antibody titers (from 1:1000 to greater than 1:1,000,000) to the ribonucleoprotein antigen fraction of extractable nuclear antigen, ~. 6 The low maternal titer to t h e Sm antigen fraction of extractable nuclear antigen, occasionally described in MCTD, and her normal D N A binding~ rarely seen in active untreated SLE, are consistent with maternal MCTD. '-~ Complete heart block as a result of focal degeneration and fibrosis of the conduction system is well described in adults with SLE, progressive systemic sclerosis, rheumatoid arthritis, polyarteritis n o d o s a , dermatomyositis, and polymyositis.~. 7.8 The pathologic changes noted in the conduction systems of neonates with CCHB born to mothers with SLE resemble those seen in adults with SLE and complete heart block? Our patient's skin lesions were
Table. Selected immunologic data Patient
Age Anti-DNP* Anti-DNA* Speekled pattern ANA* Anti-DNAt Anti-SM:~ Anti-RNP~ IgGw IgMw IgAw
Mother
1 day Neg Neg
4 mo Neg Neg
5 mo Neg Neg
1:640 920 19 < 0.45
1:80 1% Neg 1:80 500 161 207
1:20 ---
Neg Neg 1:2560 1% 1:640 1:1,000,000 5,010 155 136
Abbreviations used: Anti-DNP= anti-deoxyribonucleoprotein; antiDNA = anti-deoxyribonucleic acid; ANA = anti-nuclear antibody; SM = SM antigen fraction (RNase-insensitivefraction) of extractable nuclear antigen; RNP = ribonucleoprotein fraction (RNase-sensitive fraction) of extractable nuclear antigen. *lmmunofluorescence. t% Binding to native DNA by radioimrnunoassay(normal < 20%). :~ScrippsReference Laboratory, La Jolla, Calif. w immunodiffusion(mg/dl). similar to those of neonatal discoid and systemic lupus described by Vonderheid et a P - i n i t i a l l y scaling erythematous macules resolving with time t O hypopigmented, atrophic, telangiectatic lesions usually confined to the upper body. Although the neonatal skin lesions associated with maternal collagen-vascular disease usually resolve completely, 9 the CCHB associated with maternal collagen-vascular disease has been p e r m a n e n t in almost all reported cases. The unusual concomitant occurrence of pericarditis and CCHB in our patient, unreported in Michaelsson and Engle's comprehensive study of 599 children with CCHB, 19 suggests a common immunopathologic basis for both lesions. Six of 14 children with M C T D in Singsen's
422
The Journal of Pediatrics September 1979
Brief clinical and laboratory observations
pediatric series developed pericarditis, indicating the relatively high frequency of this complication in the pediatric age group? The transplacental passage of IgG antinuclear antibo dies suggests their etiologic role in the development of CCHB~ pericarditis, and the skin lesions of neonatal lupus in our patient. ~. 11 The decay ofth e speckled pattern A N A titer in our patient, with a half-life of approximately 30 days, parallels the established decay curves for passively acquired maternal IgG. Congenital CMV infection was initially considered in the differential diagnosis of our patient because of positive CMV titers and a serum IgM of 19 mg/dl on the first day of life. Follow-up CMV titers demonstrated the disappearance of these antibodies. The finding of a CMV titer of 1:256 in the mother corroborates a recent report demonstrating persistently elevated CMV titers in patients with MCTDY The significance of this observa"tion is unclear but may reflect the generalized hypergammaglobulinemia present in these patients. It is increasingly apparent that CCHB may result from a Wide variety of maternal collagen-vascular disorders including MCTD: clinicians should be alert to the as yet undescribed possibility of conduction disorders in the older child or adult with MCTD. REFERENCES
1. Hull D. Binns BAO. and Joyce D: Congenital heart block and Widesp!"eadfibrosis due to maternal lupus erythematosus, Arch Dis Child 41:688, 1966.
2. McCue CM, Mantakas ME, Tingelstad JB, and Ruddy S: Congenital heart block in newborns of mothers with connective tissue disease, Circulation 56:82, 1977. 3. Chameides L, Truex RC, Vetter V, et al.: Association of maternal systemic lupus eryt.hematosus with congenital complete heart block, N Engl J Med 297:1204, 1977. 4. Sharp GC, Irvin wS, May CM, et al.: Association of antibodies to ribonucleoprotein and Sm antigens wish mixed connective tissue disease, systemic lupus erythematosus and other rheumatic diseases, N Engl J Med 295:1149, 1976. 5. Singsen BH, Bernstein BH, Kornreich HK, Koster King K, Hanson V, and Tan EM: Mixed connective tissue disease in childhood, J PEDIATR90:893, 1977. 6. Notman DD, Kurata N, Tan EM: profiles of antinuclear antibodies in systemic rheumatic diseases, Ann Intern Med 83:464, 1975. 7. Bharati S, de la Fuente DJ, Kallen RJ, et al.: Conduction system in systemic lupus erythematosus with atrioventricu lar block, Am J Cardiol 35:299, 1975. 8. Schauburg HH, Nielsen SL, Yurchak PM: Heart block in polymyositis, N Engl J Med 284:480, 19'71. 9. Vonderheid EC, Koblenzer PJ, Ming PML, and Burgoon CF: Neonatal lupus erythematosus, Arch Dermatol 112:698, 1976. 10. Michaelsson M, and Engle MA: Congenital complete heart block: An international study of the natural history, Cardiovasc Clin 4:84, 1972. 11. Beck JS, Oakley CL, and Rowell NR: Transplacental passage of antinuciear antibody, Arch Dermatol 93:656, 1966. 12. Rosenthal M: Cytomegaly virus antibodies in MCTD, Arthritis Rheum 20:762, 1977.
The relationship of diapers to diaper rashes in the one-month-old infant Fred Wiener, M.D., C.M., F.R.C.P.(C), Montreal, P.Q., Canada
THE CONTRIBUTION of the diaper to rashes in the diaper area has b e e n a point Of contention for many years. The layette for babies in the early decades consisted of various cloths and Y~soakers." The last was a wool overgarment simiIar in style and function to present-day plastic pants? In the 1930s and 40s, rubber pants became popular but were available in larg e sizes only. The smaller baby wore diapers with or without woven or knitted pants, and was usually positioned on a quilted pad. All of the above techniques drew urine away from the baby's diaper area and allowed air to enter. Repri m address." 6212 Clanranald A re, Montreal, P. Q., Canada H 3 X 2T3.
During the 1950s, plastic pants in sizes suitable for infants under three months became popular. It is probably more than coincidence that monilial diaper ras h was recognized as an important entity at that time. ~ Burgoon and Wright ~ listed several predisposing and activating factors, including ',maceration caused by continuous contact ,with a wet diaper and increased by the moist heat produce d by an impervious rubber cover." Thus, the technologic advance of occluding the small baby's bottom with impervious rubber pants may have led to the considerable frequency of the monilial variety of diaper rash. The convenience and watertight properties of the disposable plasticized paper diape r led to its widespread
0022=3476/79/090422 +03500.30/0 9 1979 The C. V. Mosby Co.