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KMID : 0367419650080030025
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Journal of Korean Pediatric Society
1965 Volume.8 No. 3 p.25 ~ p.30
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Histiocytosis X
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洪淳子/Hong, Soon Za
李鴻烈/鄭性煥/尹惠善/Lee, Hong Yul/Chung, Sung Whan/Yoon, Hae Sun
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Abstract
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This report presents the three cases of Histiocytosis X which were experienced at St. Mary´s Hospital during the past two years, and includes the discussion
of the interrelationship of these diseases. 3
(Case 1.) L.H.S., an 1 12 year-old girl began com-
plaining of bilateral purulent otitis media and low
grade fever at the age of nine months. One month later a scaly erythematous, papular and . hemorrhagic rashes developed over the scalp, neck and trunk. During the ensuing three months she was treated symptomatically, but splenomegaly, severe anemia, lytic bone lesion on the right frontal of skull bone and congestive failure developed. She died six months after the occurence of her initial symptoms. The biopsy findings were similar to the microscopic features of Nonlipid Histiocytosis, namely Letterer-Siwe Disease.
(Case 2. ) K.H.S., a 7 year-old girl showed initial symptoms, such as fever and headache at the age of 4 years. Four months later she developed polyuria, polyphasia, exophthalmus and lytic lesions of the skull, iliac, proximal end of femurs and right 4th, 5th ribs, lytic defect on the left external auditory canal. The biopsy findings were similar to features of Hand -Schuller-Christian´s Disease.
(Case 3.) H.K.N., a 7 year-old girl, started complaining of polyuria and polyphasia about three months before she was admitted. But she did not has any other clinical symptoms. Water intake was over 6, 000 ml a day and daily urinary volume was near 6, 000 ml. Semicircular solitary lytic lesion 10 x 7 cm on the lateral and parietal bone was found in skull x-ray examination. The biopay findings revealed Eosinophilic Granuloma.
It is interesting that lytic defects in the submaxillar sinus or external auditory canal were found and two of them had the episodes of acute or chronic otitis media.
Case 3. had symptoms of diabetes insipidus and large single lytic lesion of the skull bone, which clinic-ally suggested the transitional form between Hand-Schuller-Christians Disease and Eosinophilic Granuloma, but the biopsy showed the feature of Eosinophilic Granuloma. It is considered that these evidences are helpful in understanding the nature of these diseases.
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