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KMID : 0363219700080020073
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Korean Journal of Dermatology
1970 Volume.8 No. 2 p.73 ~ p.77
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Incontinentia Pigmenti (Report of 4 Cases in a Family)
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趙啓恩/Cho, Kye Eun
朴旭和/吳柱益/禹泰河/Bark, Wook Hwa/Oh, Joo Ik/Woo, Tae Ha
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Abstract
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Four cases of incontinentia pigmenti (Bloch-Sulzberger type) in a mother and three her daugL:er were presented and literatures were reviewed
In all three daughters vesicles appeared on toes at three weeks after birth and changed to pigmented patches surrounded by erythematous verrucous margin, which were then gradually spread to whole body. Pigmentation is dirty, irregular and bizarr. Severe itching sensation was complained and vesicles or bullae were formed easily due to scratching and external trauma. In mother, onset of the disease was not clear but generalized dirty pigmentation is ´showed. Dentition was delayed in 3rd daughter (Dental eruption was begun at 10 month old and 11 teeth was shoes at now, 2 year old).
Histopathological findings are compatible with incontinentia pigmenti and epidermo-dermal separation is observed in a experimentally induced bullae, indicating a characterized epidermolysis bullosa.
Strict limitation in the occurence of disease in females of one family, accompanying with epidermolysis bullosa simplex is unusual and interesting in genetical aspects and pathogenesis.
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