Porokeratotic eccrine ostial and dermal duct naevus (PEODDN) is a rare, benign hamartomatous malformation involving the eccrine sweat duct. The existence of filiform keratinous plugs that represent cornoid lamellae overlying dilated infundibula of eccrine ducts is a distinctive feature and the prese
Systematized porokeratotic eccrine and hair follicle naevus: report of a case and review of the literature
β 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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β¦ 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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## Abstract ## Background. Eccrine spiradenocarcinoma is a rare dermal appendage carcinoma believed to arise from transformation of a longβstanding benign spiradenoma. This tumor demonstrates highly malignant biologic behavior with a high recurrence rate, frequent lymph node metastases, and overal