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Year : 2008  |  Volume : 74  |  Issue : 5  |  Page : 485--486

Pachyonychia congenita with woolly hair in a ten month old infant

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran

Correspondence Address:
Fatemeh Moeineddin
Department of Dermatology, Razi Hospital, Vahdat Eslami Square, Tehran-119 960
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0378-6323.44306

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A 10-month-old female presented with severe progressive wedge-shaped thickening and discoloration of all twenty nails. Further evaluations revealed palmoplantar keratoderma along with recurrent acral blisters causing residual crusted ulcers which were present during the past six months. Other findings included scalp kinky hair and dental caries. Past medical and family history had remarkable findings such as natal teeth and similar skin lesions in her older brother since his infancy. The patients' clinical presentations and history are compatible with pachyonychia congenita presenting with concomitant features of both subtypes 1 and 2.


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Online since 15th March '04
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