2010
DOI: 10.1016/j.jaad.2010.01.017
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The group of epidermal nevus syndromes

Rudolf Happle
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Cited by 206 publications
(177 citation statements)
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References 234 publications
(220 reference statements)
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“…1 Nevi associated with ENSs follow the lines of Blaschko, a notable exception to which is the CHILD syndrome, in which lateralization pattern reflects the functional origin of the postzygotic modulation of the inherited mutation. 1 Nevus sebaceous occurring as a part of nevus sebaceous syndrome may be differentiated histologically, by the presence of hyperplastic sebaceous glands. 1 Coexistence of nevus sebaceous and nevus philus may be seen in phacomatosis pigmentokeratotica.…”
Section: Discussionmentioning
confidence: 96%
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“…1 Nevi associated with ENSs follow the lines of Blaschko, a notable exception to which is the CHILD syndrome, in which lateralization pattern reflects the functional origin of the postzygotic modulation of the inherited mutation. 1 Nevus sebaceous occurring as a part of nevus sebaceous syndrome may be differentiated histologically, by the presence of hyperplastic sebaceous glands. 1 Coexistence of nevus sebaceous and nevus philus may be seen in phacomatosis pigmentokeratotica.…”
Section: Discussionmentioning
confidence: 96%
“…1 Nevus sebaceous occurring as a part of nevus sebaceous syndrome may be differentiated histologically, by the presence of hyperplastic sebaceous glands. 1 Coexistence of nevus sebaceous and nevus philus may be seen in phacomatosis pigmentokeratotica. Ipsilateral cataract is the diagnostic clue in nevus comedonicus syndrome, whereas linear nevus covered with soft white hair distinguishes Angora hair nevus from other epidermal nevi (EN).…”
Section: Discussionmentioning
confidence: 99%
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“…In LEN marked papillomatosis, hyperkeratosis, and acanthosis with elongation of rete ridges in psoriasiform pattern is noted. 3 The observation of epidermolytic hyperkeratosis on biopsy should prompt genetic counseling. The often devastating disfigurement of epidermolytic hyperkeratosis (EHK) results from mutations in keratins 1 and 10.…”
Section: Discussionmentioning
confidence: 99%
“…1,2 Currently, four major different types of ENS are recognized: linear sebaceous nevus, linear nevus comedonicus, linear epidermal nevus, and inflammatory linear verrucous epidermal nevus (ILVEN). 3 ENS is likely a continuum, as many authors include a wide-array of various other syndromes under its mantle. The presence of epidermal nevi should alert the clinician to possible abnormalities in the underlying organ systems.…”
Section: Case Reportmentioning
confidence: 99%