Phacomatosis Pigmentokeratotica | Zendy

Gianluca Tadini | Zendy; Lucia Restano | Zendy; Ricardo Gonzáles-Pérez | Zendy; M. Antonia Gonzáles-Enseñat | Zendy; M. Asunción Vincente-Villa | Zendy; Stefano Cambiaghi | Zendy; Paolo Marchettini | Zendy; Massimo Mastrangelo | Zendy; Rudolf Happle | Zendy

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Phacomatosis Pigmentokeratotica

Author(s) -

Gianluca Tadini,

Lucia Restano,

Ricardo Gonzáles-Pérez,

M. Antonia Gonzáles-Enseñat,

M. Asunción Vincente-Villa,

Stefano Cambiaghi,

Paolo Marchettini,

Massimo Mastrangelo,

Rudolf Happle

Publication year - 1998

Publication title -

archives of dermatology

Language(s) - English

Resource type - Journals

eISSN - 1538-3652

pISSN - 0003-987X

DOI - 10.1001/archderm.134.3.333

Subject(s) - medicine , dermatology , nevus , genodermatosis , hyperhidrosis , dysesthesia , pathology , biology , genetics , melanoma , cancer research , anesthesia , gene

The epidermal nevus syndromes include different diseases that have the common feature of mosaicism. One of these has been recently identified and named phacomatosis pigmentokeratotica, in analogy to phacomatosis pigmentovascularis. It is characterized by an organoid nevus with sebaceous differentiation, a speckled-lentiginous nevus, and other associated anomalies. It has been hypothesized that this syndrome is caused by a particular genetic mechanism known as the twin-spot phenomenon.

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