Which clinical history findings are characteristic of less common variants of porokeratosis?

Updated: Oct 09, 2020
  • Author: Amarateedha Prak LeCourt, MD; Chief Editor: William D James, MD  more...
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Answer

Porokeratosis ptychotropica presents with inflammatory keratotic plaques with histopathologic foci of cornoid lamellae on the buttocks or genitals, and may be mistaken for psoriasis. [65, 66]

Follicular porokeratosis is described as an eruptive papular porokeratosis in the setting of DSP. Porokeratosis may occur syndromically with craniosynostosis and anal anomalies (CAP syndrome). [67]

Porokeratotic adnexal osteal nevus is the name proposed to incorporate porokeratotic eccrine ostial and dermal duct nevus (PEODDN) and porokeratotic eccrine and hair follicle nevus (PEHFN). It is considered a congenital lesion, usually developing during infancy or childhood. Lesions consist of hyperkeratotic papules and plaques, and occasionally punctate pits filled with a comedolike keratin plug. It develops typically on the distal extremities, although proximal extremities, trunk, and face may be involved. During the neonatal period, the lesions may initially appear as erosions. Lesions are often aligned along the lines of Blaschko and may extend into nailbeds. See the image below.

A young boy with a linear lesion of porokeratotic A young boy with a linear lesion of porokeratotic eccrine ostial and dermal duct nevus extending onto the nailbed, causing pterygium formation.

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