852
Letters to the Editor
CASE REPORT
Patient J.Y., a 3t/z-year-old male, was admitted with marked pallor and gross hematuria. Ten days earlier he had been exposed to varicella. Eight days before admission he developed diarrhea and vomiting. Blood pressure was 160/110. Hemoglobin (Hgb) was 7.1 gm/dl, platelet count 64,000/mm 3, and reticulocyte count was 6.2%. Many fragmented red blood cells (RBC) were seen on smear. Urinalysis contained 4 + protein. Microscopic analysis revealed 50 to 100 RBC/high-power field and RBC casts. A diagnosis of hemolytic uremic syndrome was made and he was treated with antihypertensive medication. He recovered and was well for the next 13 months, when he was again exposed tO Varicella. Two weeks tater he developed rhinorrhea, epistaxis, petechiae, and ecchymoses. Blood pr~essure was normal. Laboratory data included: Hgb 11.4 gm/dl, hematocrit 32%, platelet count 1,000/mm 3. RBC morphology, urinalysis, prothrombin time, and partial thromboplasin time, total hemolytic complement and quantitative immunoglobulins were all normal. Bone marrow showed mature megakaryocytes. The diagnosis of ITP was made. On the third hospital day he developed fever and a varicelliform rash. Giant cells were seen in scrapings from the vesicles. He recovered spontaneously and has been well for two years since this illness. COMMENT
This child had typical HUS followed in 13 months by typical ITP. Both illnesses occurred after exposure to varicella. Unfortunately, neither serologic or virologic data exist to assess the occurrence of varicella with the first illness. Evidence of variCella infection occurred with the second illness. Varicella has been reported to recur in other patients? The occurrence of ITP and HUS in a single patient is a unique observation, not previously reported so far as we can ascertain. Stanley A. Mendoza, M.D. Billy B. Sellers, Jr., M.D. William R. Griswold, M.D. University of California, San Diego Department of Pediatrics School of Medicine La Jolla, CA 92093
The Journal of Pediatrics November 1977
development. The new "subunit" of chondrodysplasia punctata has to be accepted. According to the present report it might be not at all rare. Yet, it was not as completely neglected as the Australian study supposed. My 1950 study arrived at rather similar conclusions as the recent paper from Melbourne. Walter Swoboda, M.D. Professor of Pediatrics Ludwig Boltzmann InstitUt f paediat. Endokrinologie Sehrankenberggasse 31 A- 1100 Vienna, Austria REFERENCES
1. Sheffield LJ, Danks DM, Mayne V, and Hutchinson LA: Chondrodysplasia punctata-23 cases Of a mild and relatively common variety, J PEDIATR 89:916, 1976. 2. Swoboda W.: Beitrag zur Chondrodystrophia calcificans congenita. (Ein abortiver Fall dieser Erkrankung), Ann Paediatr 175:322, 1950.
Reply To the Editor: We tried to indicate that a number of individual cases of this syndrome had been described-enough to render listing pointless. Our contribution was to note the relatively high frequency and benign nature of this variant of chondrodysplaSia p unctata and to commence the task of defining the full range of the Syndrome and of searching for its cause (or causes). D.M. Danks, M.D. Royal Children's Hospital Melbourne, 3052, Australia
Pylorie atresia and epidermolysis bullosa
REFERENCE
1. Gordon JE: Chickenpox: An epidemiologic review, Am J Med Sci 244:362, 1962.
Chondrodysplasia punctata To the Editor: The recent paper On chondrodysplasia punctata 1 is a very valuable addition to the list of subcategories of this puzzling syndrome. Type and number of cases reported here and "neglected in the earlier studies" are impressive indeed. I published a very similar case many years ago. 2 The appearance of the face as well as the irregular punctate calcification of the calcaneum was quite the same as in the present collection of'patients. In addition, my patient was male and slightly retarded in height and in mental
To the Editor: Korber and Glasson' reported in THE JOURNAL in April, 1977, an unusual association of pyloric atresia and epidermolysis bullosa in two unrelated Lebanese infants. In February, 1977, we treated a neonate With the same syndrome. A male Turkish infant, with a birth Weight of 1,520 gm, was born after a pregnancy complicated by hydramnios. Two other siblings are alive and well. From birth the child developed bullae on feet and hands; a staphylococcal dermatitis was suspected, and he was treated with antibiotics. Cultures from the bullae were negative. Blistering of the skin continued, and a clinical diagnosis of epidermolysis bull0sa, probably of the dystrophic recessive type, was made. There was continuous vomiting and a roentgenogram of the abdomen showed the "single bubble" sign: no intestinal air was demonstrable. On the fourth day, a membranous diaphragmatic
Letters to the Editor
CASE REPORT
Patient J.Y., a 3t/z-year-old male, was admitted with marked pallor and gross hematuria. Ten days earlier he had been exposed to varicella. Eight days before admission he developed diarrhea and vomiting. Blood pressure was 160/110. Hemoglobin (Hgb) was 7.1 gm/dl, platelet count 64,000/mm 3, and reticulocyte count was 6.2%. Many fragmented red blood cells (RBC) were seen on smear. Urinalysis contained 4 + protein. Microscopic analysis revealed 50 to 100 RBC/high-power field and RBC casts. A diagnosis of hemolytic uremic syndrome was made and he was treated with antihypertensive medication. He recovered and was well for the next 13 months, when he was again exposed tO Varicella. Two weeks tater he developed rhinorrhea, epistaxis, petechiae, and ecchymoses. Blood pr~essure was normal. Laboratory data included: Hgb 11.4 gm/dl, hematocrit 32%, platelet count 1,000/mm 3. RBC morphology, urinalysis, prothrombin time, and partial thromboplasin time, total hemolytic complement and quantitative immunoglobulins were all normal. Bone marrow showed mature megakaryocytes. The diagnosis of ITP was made. On the third hospital day he developed fever and a varicelliform rash. Giant cells were seen in scrapings from the vesicles. He recovered spontaneously and has been well for two years since this illness. COMMENT
This child had typical HUS followed in 13 months by typical ITP. Both illnesses occurred after exposure to varicella. Unfortunately, neither serologic or virologic data exist to assess the occurrence of varicella with the first illness. Evidence of variCella infection occurred with the second illness. Varicella has been reported to recur in other patients? The occurrence of ITP and HUS in a single patient is a unique observation, not previously reported so far as we can ascertain. Stanley A. Mendoza, M.D. Billy B. Sellers, Jr., M.D. William R. Griswold, M.D. University of California, San Diego Department of Pediatrics School of Medicine La Jolla, CA 92093
The Journal of Pediatrics November 1977
development. The new "subunit" of chondrodysplasia punctata has to be accepted. According to the present report it might be not at all rare. Yet, it was not as completely neglected as the Australian study supposed. My 1950 study arrived at rather similar conclusions as the recent paper from Melbourne. Walter Swoboda, M.D. Professor of Pediatrics Ludwig Boltzmann InstitUt f paediat. Endokrinologie Sehrankenberggasse 31 A- 1100 Vienna, Austria REFERENCES
1. Sheffield LJ, Danks DM, Mayne V, and Hutchinson LA: Chondrodysplasia punctata-23 cases Of a mild and relatively common variety, J PEDIATR 89:916, 1976. 2. Swoboda W.: Beitrag zur Chondrodystrophia calcificans congenita. (Ein abortiver Fall dieser Erkrankung), Ann Paediatr 175:322, 1950.
Reply To the Editor: We tried to indicate that a number of individual cases of this syndrome had been described-enough to render listing pointless. Our contribution was to note the relatively high frequency and benign nature of this variant of chondrodysplaSia p unctata and to commence the task of defining the full range of the Syndrome and of searching for its cause (or causes). D.M. Danks, M.D. Royal Children's Hospital Melbourne, 3052, Australia
Pylorie atresia and epidermolysis bullosa
REFERENCE
1. Gordon JE: Chickenpox: An epidemiologic review, Am J Med Sci 244:362, 1962.
Chondrodysplasia punctata To the Editor: The recent paper On chondrodysplasia punctata 1 is a very valuable addition to the list of subcategories of this puzzling syndrome. Type and number of cases reported here and "neglected in the earlier studies" are impressive indeed. I published a very similar case many years ago. 2 The appearance of the face as well as the irregular punctate calcification of the calcaneum was quite the same as in the present collection of'patients. In addition, my patient was male and slightly retarded in height and in mental
To the Editor: Korber and Glasson' reported in THE JOURNAL in April, 1977, an unusual association of pyloric atresia and epidermolysis bullosa in two unrelated Lebanese infants. In February, 1977, we treated a neonate With the same syndrome. A male Turkish infant, with a birth Weight of 1,520 gm, was born after a pregnancy complicated by hydramnios. Two other siblings are alive and well. From birth the child developed bullae on feet and hands; a staphylococcal dermatitis was suspected, and he was treated with antibiotics. Cultures from the bullae were negative. Blistering of the skin continued, and a clinical diagnosis of epidermolysis bull0sa, probably of the dystrophic recessive type, was made. There was continuous vomiting and a roentgenogram of the abdomen showed the "single bubble" sign: no intestinal air was demonstrable. On the fourth day, a membranous diaphragmatic
