Segmental Neurofibromatosis Clinically Appearing as a Nevus Spilus

Finley, Eric M.; Kolbusz, Robert V.

doi:10.1111/j.1365-4362.1993.tb01473.x

Cited by 21 publications

(12 citation statements)

References 17 publications

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“…Epidemiological studies reveal that females are affected twice as often as males (8). The age of onset falls into a bimodal distribution, with peaks at 10-30 years and 50-70 years (9). The right side of the body is more commonly affected than the left side (4:3), and approximately 6% of cases are bilateral (10).…”

Section: Discussionmentioning

confidence: 98%

Segmental Neurofibromatosis

Niiyama¹,

Satoh²,

Kaneko³

et al. 2005

Acta Dermato-Venereologica

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Sir, Neurofibromatosis (NF) is a heterogeneous disorder clinically characterized by the presence of neurofibromas, multiple café-au-lait spots, intertriginous freckles and Lisch nodules (pigmented iris hamartomas). We describe an unusual case of NF with cutaneous neurofibromas on the waist. The distribution was unilateral on the anterior aspect of the waist and bilateral on the posterior aspect. CASE REPORTA 68-year-old Japanese woman was referred to our department for evaluation of skin lesions of her waist. At the age of 30, she had noticed gradually increasing numbers of these lesions, limited to the same cutaneous areas. No other members of her family or close relatives exhibited a similar condition or abnormal pigmentation.Her past history was unremarkable except for a colon carcinoma. Clinical examination of the skin revealed numerous, soft, reddish nodules measuring 3-8 mm in diameter, showing a segmental arrangement on the posterior aspect bilaterally and on the left side with sharp demarcation at the mid-line on the anterior aspect ( Fig. 1). They were pruritic and minimally tender on palpation. A biopsy specimen of the nodule showed typical features of neurofibroma. No café-au-lait spots or Lisch nodules were detected. Complete blood cell count and chemical studies were normal, and chest X-ray and nuclear magnetic resonance imaging of the head were normal. DISCUSSIONBecause of the great variability of its clinical presentation, Riccardi classified NF into eight categories based Acta Derm Venereol 85

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Section: Discussionmentioning

confidence: 98%

Segmental Neurofibromatosis

Niiyama¹,

Satoh²,

Kaneko³

et al. 2005

Acta Dermato-Venereologica

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“…55 Some of the features of this neurocristopathy are reminiscent of NF1, including the CALM-like background, development of lentigines, and associated neurologic abnormalities (referred to as the ''SLN syndrome''). 56 Pigmented lesions that likely represented SLN have been classified as NF1 when neurotized nevi have developed as speckles 57,58 or when musculoskeletal abnormalities related to SLN syndrome have been present. 59 In addition to reports of patients with segmental lentiginosis together with segmental NF1, 60-62 SLN spatially associated with segmental lentiginosis plus segmental NF1 and with segmental NF1 alone have been described.…”

Section: Discussionmentioning

confidence: 99%

Pigmented plexiform neurofibroma: Distinction from a large congenital melanocytic nevus

Schaffer¹,

Chang

Kovich

et al. 2007

Journal of the American Academy of Dermatology

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“…The terminology of SLN or nevus spilus is confusing and SLN is sometimes misdiagnosed as PUL [19]. In the past, such cases have sometimes been incorrectly categorized as examples of segmental NF1 [20, 21], whereas the correct diagnosis would have been SLN [22]. When this skin disorder is associated with neurological or other extracutaneous anomalies, there is still no reason to think of segmental NF1.…”

Section: Discussionmentioning

confidence: 99%

Partial Unilateral Lentiginosis with Ipsilateral Lisch Nodules and Axillary Freckling

Chen¹,

Fan²,

Happle³

2004

Dermatology

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A 9-year-old Taiwanese girl had an unusual combination of multiple lentigines clustered on her left face, 3 café-au-lait spots on her left arm and ipsilateral axillary freckling. Two Lisch nodules of the iris were recognized in the left eye but none in the right eye, which is why we established a diagnosis of segmental neurofibromatosis 1. No family member had any sign of neurofibromatosis 1. Moreover, the patient had a large speckled lentiginous nevus involving the left side of her trunk, and her father showed a similar skin disorder on his left buttock. These lesions were taken to be unrelated to the patient’s segmental neurofibromatosis 1. This case provides further evidence in favor of the concept that partial unilateral lentiginosis represents a mosaic manifestation of type 1 neurofibromatosis.

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Segmental Neurofibromatosis Clinically Appearing as a Nevus Spilus

Cited by 21 publications

References 17 publications

Segmental Neurofibromatosis

Segmental Neurofibromatosis

Pigmented plexiform neurofibroma: Distinction from a large congenital melanocytic nevus

Partial Unilateral Lentiginosis with Ipsilateral Lisch Nodules and Axillary Freckling

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