Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature.
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.
Kroumpouzos
G
G
Department of Dermatology, Boston University School of Medicine Boston, MA 02118, USA.
Stefanato
C M
CM
Wilkel
C S
CS
Bogaars
H
H
Bhawan
J
J
D000293:Adolescent; D000506:Alopecia Areata / Q000473:pathology; D005260:Female; D018859:Hair Follicle / Q000473:pathology; D006801:Humans; D009506:Nevus / Q000473:pathology*; D017499:Porokeratosis / Q000473:pathology*; D012878:Skin Neoplasms / Q000473:pathology*