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Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature
Author(s) -
Kroumpouzos G.,
Stefanato C.M.,
Wilkel C.S.,
Bogaars H.,
Bhawan J.
Publication year - 1999
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1999.03210.x
Subject(s) - dermatology , hair follicle , histopathology , medicine , differential diagnosis , pathology , parakeratosis
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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