Back issues No.2 - 1991 / Original Study  

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Incontinentia Pigmenti, Analysis of 16 Cases
Hsiu-Chin Chen, Yang-Jyh Lin
Dermatol Sinica 9: 75-84, 1991

Sixteen cases of incontinentia pigmenti seen at Mackay Memorial Hospital in the last twenty years were analysed. Six cases (37.5%) had definite familial history. Ten cases (62.5%) presented three stages of characteristic skin manifestations: vesiculobullous, verrucous and pigmentary stages. No vesiculobullous stage was noted in 3 cases (18.75%), and 3 cases presented only in the pigmentary stage without history of earlier lesion. These 16 cases were all female, and in most of them, skin lesions were noted at birth or soon after birth. Other associated defects included neurological, musculoskeletal, cardiovascular, ocular, dental and hair abnormalities. Neurological abnormalities had the highest incidence - 44%(7 cases).

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