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Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature

✍ Scribed by G. Kroumpouzos; C.M. Stefanato; C.S. Wilkel; H. Bogaars; J. Bhawan


Publisher
John Wiley and Sons
Year
1999
Tongue
English
Weight
422 KB
Volume
141
Category
Article
ISSN
0007-0963

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✦ Synopsis


We report a unique case of a congenital keratinocytic naevus associated with severe alopecia, onychodysplasia and palmoplantar involvement in a 13-year-old girl. The lesions, consisting of scaly, spinous and verrucous papules and plaques, mainly followed Blaschko's lines and have remained unchanged since birth. The predominant histopathological picture was that of a column of parakeratosis overlying the eccrine ostia and hair follicles. This is the first case of a systematized keratinocytic naevus characterized by histopathology of eccrine and hair follicle porokeratosis and a widespread bilateral involvement. This may be a distinct entity to be included in the differential diagnosis of linear, hyperkeratotic dermatoses. We suggest its classification as systematized porokeratotic eccrine and hair follicle naevus.


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