Bilateral segmental neurofibromatosis with partial unilateral lentiginosis

Wong, Sai Siong

doi:10.1111/j.1365-2133.1997.tb14949.x

Cited by 22 publications

(15 citation statements)

References 21 publications

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“…Caf e-au-lait (milk and coffee) spots and neurofibromas are the defining features. [10][11][12] Infantile capillary hemangioma was observed in 10 patients. NF1 was observed in 15 patients.…”

Section: Resultsmentioning

confidence: 99%

The spectrum of genodermatoses and congenital cutaneous conditions in northern Ethiopia

Dassoni¹,

Morrone

Padovese³

2014

Int J Dermatology

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Reports of congenital diseases in Africa are scanty, probably because of their rarity, the lack of knowledge among health workers, and the difficult political and social situation in different African countries. We describe here the spectrum of genetic and rare congenital cutaneous conditions encountered at the Italian Dermatological Center of Ayder referral hospital of Mekele, Ethiopia, over a 3-year period. All patients attending the Italian Dermatological Center were registered in a database, and medical records of genetic and congenital disorders diagnosed from January 2008 to December 2010 were retrospectively analyzed. Over the total, 24 different genetic and congenital disorders affecting 122 individuals (0.4% of the total case load) were observed. In our case series, we did not report any patient affected by albinism, in contrast with literature from other African countries. To our knowledge, this is the first report from northern Ethiopia. A brief update on the commonest disorders is included.

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Section: Resultsmentioning

confidence: 99%

The spectrum of genodermatoses and congenital cutaneous conditions in northern Ethiopia

Dassoni¹,

Morrone

Padovese³

2014

Int J Dermatology

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“…Phacomatosis pigmentovascularis may be associated with a speckled lentiginous nevus [9], but this particular type of nevus is quite flat and does never show melanocytic papules. Partial unilateral lentiginosis is presently taken as a mosaic manifestation of neurofibromatosis 1 [10]. Again, partial unilateral lentiginosis that has sometimes been confused with speckled lentiginous nevus [5, 6]is never superimposed by melanocytic papules.…”

Section: Discussionmentioning

confidence: 99%

Speckled Lentiginous Nevus Syndrome: Report of a Further Case

Vente

Neumann²,

Bertsch³

et al. 2004

Dermatology

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A 42-year-old man had a large speckled lentiginous nevus on the left side of his trunk. The involved area was painful when touched and paresthetic. Moreover, the ipsilateral half of his body showed a pronounced hyperhidrosis. This case can be categorized as a typical example of speckled lentiginous nevus syndrome, a recently recognized phenotype characterized by a speckled lentiginous nevus of the papular type and ipsilateral neurological abnormalities in the form of dysesthesia, muscular weakness or hyperhidrosis. Speckled lentiginous nevus syndrome represents a mosaic phenotype. Most likely it originates from loss of heterozygosity occurring in a heterozygous embryo at an early developmental stage.

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“…Several reports have described unilateral and regional lentiginosis with different cutaneous and systemic abnormalities. The association of PUL with nevus depigmentosus (4), segmental neurofibromatosis (5, 6), blue nevi (7), as well as sickle cell anemia (8), mental retardation (9), and seizures (10), among others, has been reported.…”

Section: Commentmentioning

confidence: 99%