The Epidermal Nevus Syndrome: Case Report and Review

Goldberg, Leonard Harry; Couins, Scott A.B.; Siegel, Daniel M.

doi:10.1111/j.1525-1470.1987.tb00749.x

Cited by 29 publications

(19 citation statements)

References 21 publications

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“…In the newborn, a uniformly tannish-brown macule without speckles may be seen, whereas the dark papules develop later [1, 8, 12, 13, 27, 31,42,43,44,45,46,48,49,50,51]. In some cases, however, the dark papules may already be noted at birth [1, 8, 14, 30]. The arrangement of papular SLN always corresponds to a checkerboard pattern although this may not be conspicuous when the lesion involves only a small area of the body.…”

Section: Papular Slnmentioning

confidence: 99%

“…Papular SLN may occur as an isolated lesion [1, 4, 5, 8, 13, 14, 43, 46, 49] or as a component of phacomatosis pigmentokeratotica [1,27,28,29,30,31,32, 50, 51,55,56,57]or of SLN syndrome (table 4) [33]. Phacomatosis pigmentokeratotica has been proposed to represent an example of didymosis (twin spotting) [26].…”

Section: Papular Slnmentioning

confidence: 99%

“…Occasionally, the arrangement has erroneously been called ‘zosteriform’ [4,15,16,17], although the distribution is definitely not dermatomal. Sometimes, SLN may be part of complex birth defects such as phacomatosis pigmentovascularis [18,19,20,21,22,23,24,25], phacomatosis pigmentokeratotica [26,27,28,29,30,31,32], or SLN syndrome [33]. …”

Section: Introductionmentioning

confidence: 99%

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Two Distinct Types of Speckled Lentiginous Nevi Characterized by Macular versus Papular Speckles

Cruz

Happle²

2006

Dermatology

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Background: Speckled lentiginous nevus (SLN; synonym: nevus spilus) is a darkly spotted light-brown macule that mostly occurs as an isolated lesion of rather limited dimensions but sometimes may involve large areas of the body. So far, this skin disorder has been considered to represent one clinical entity. Objective: Because SLN is occasionally associated with complex birth defects such as phacomatosis pigmentovascularis, phacomatosis pigmentokeratotica, or SLN syndrome, we tested our idea that two different types of SLN may exist, each one associated with a particular syndrome. Methods: A review of case reports on SLN published during the years 1970–2004 was performed. Results: This evaluation of cases provided evidence that two different types of SLN exist, in the form of macular versus papular SLN, each one being related to a specific syndrome. Macular SLN is characterized by a tannish-brown background with darker flat speckles. The distribution of speckles is rather even and resembles a polka-dot pattern. Histopathologically, this type of SLN is characterized by what has been called a ‘jentigo’ pattern in the darker speckles and by some nests of melanocytes at the dermoepidermal junction at the tips of the papillae, whereas the background pigmentation shows the microscopical features of a lentigo. Papular SLN is characterized by a light-brown macule superimposed by multiple melanocytic nevi in the form of papules or nodules that show a more uneven distribution reminiscent of a star map. Small dark macules may likewise be present. Histopathologically, the papular component consists of dermal or compound melanocytic nevi. A separation of the two types of SLN is important because our analysis showed that macular SLN is a hallmark of a particular type of phacomatosis pigmentovascularis, whereas papular SLN is typically present in phacomatosis pigmentokeratotica as well as in SLN syndrome. Conclusions: Macular SLN and papular SLN appear to be two distinct cutaneous entities. This dichotomy may turn out to be important when such nevi will be analyzed at the molecular level.

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Section: Papular Slnmentioning

confidence: 99%

Section: Papular Slnmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Two Distinct Types of Speckled Lentiginous Nevi Characterized by Macular versus Papular Speckles

Cruz

Happle²

2006

Dermatology

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“…1 These cases have been given various names, such as epidermal nevus syndrome, sebaceous nevus syndrome, FeuersteinMims neuroectodermal syndrome, and "nevus on a nevus." [7][8][9][10][11][12] In patient 2, a biopsy specimen obtained from the epidermal nevus showed no signs of adnexal hyperplasia. We are unable to say, however, whether such organoid differentiation is absent in all parts of the epidermal nevus.…”

Section: Commentmentioning

confidence: 99%

Phacomatosis Pigmentokeratotica

Tadini¹,

Restano²,

Gonzáles-Pérez³

et al. 1998

Arch Dermatol

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“…However, we recently carried out a systematic review on segmental SLN (unpublished data) and found that only 84 out of the 281 cases reported in the literature (29.9%) were part of a syndrome, and only 2 (0.7%) were associated with angiomas (and cutaneous ones) [4,5] and that in the vast majority of syndromic cases, both SLN and, if present, the associated nevus (nevus roseus or nevus sebaceus) were extensive and often involving both sides of the body.…”

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confidence: 99%

Speckled lentiginous nevus: sometimes, but not always, part of a syndrome

Torchia

Schachner

2011

Neurol Sci

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We read with great interest the recent paper by Boncoraglio et al. [1]. They reported on an adult female patient affected by a large speckled lentiginous nevus (SLN) associated with an intracranial cavernoma and concluded that she had either phacomatosis pigmentovascularis or SLN syndrome [1]. We believe that such a differential diagnosis, and the fact that SLN was part of a syndrome, may not be correct. First, according to current knowledge, the only type of phacomatosis pigmentovascularis featuring a SLN is phacomatosis spilorosea, which is characterized by the association of a macular SLN with nevus roseus [2]. Instead, in the case by Boncoraglio et al., no nevus roseus was present [1]. Second, SLN syndrome is characterized by a papular SLN associated with ipsilateral peripheral nerve, neuromuscular and/or central nervous system disturbances [3]. On the other hand, Boncoraglio et al. did not describe any of these anomalies if we exclude those mechanically induced by the intracranial cavernoma or its surgical management [1]. Third, the only other known syndrome featuring a SLN, i.e., phacomatosis pigmentokeratotica, had to be ruled out because of the lack of an associated nevus sebaceus.The fact that the intracranial cavernoma was ipsilateral and spacially close to the SLN may suggest that both lesions were determined by the migration of abnormal cell clones at some point during ontogenesis and thus were somehow pathogenically related. However, we recently carried out a systematic review on segmental SLN (unpublished data) and found that only 84 out of the 281 cases reported in the literature (29.9%) were part of a syndrome, and only 2 (0.7%) were associated with angiomas (and cutaneous ones) [4,5] and that in the vast majority of syndromic cases, both SLN and, if present, the associated nevus (nevus roseus or nevus sebaceus) were extensive and often involving both sides of the body.On the basis of these arguments, it is our opinion that there is not enough ground to call for a (new) syndrome and therefore, that Boncoraglio et al. dealt with an isolated maculopapular SLN randomly associated with intracranial cavernoma. References1. Boncoraglio GB, Parati EA, Ciceri E, Rinaldi R, Capella GL (2010) Intracranial cavernoma and speckled lentiginous nevus: extending the spectrum of phakomatoses? Neurol Sci 31:841-844 2. Happle R (2005) Phacomatosis pigmentovascularis revisited and reclassified. Arch Dermatol 141:385-388 3. Torchia D (2010) More on speckled lentiginous nevus syndrome. Dermatology 221:324-325 4. Brufau C, Moran M, Armijo M (1986) Naevus sur naevus: A propos de 7 observations, trois associees a d'autres dysplasies, et une a un melanome malin invasif. Ann Dermatol Venereol 113:409-418 5. Goldberg LH, Collins SA, Siegel DM (1987) The epidermal nevus syndrome: case report and review.

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The Epidermal Nevus Syndrome: Case Report and Review

Cited by 29 publications

References 21 publications

Two Distinct Types of Speckled Lentiginous Nevi Characterized by Macular versus Papular Speckles

Two Distinct Types of Speckled Lentiginous Nevi Characterized by Macular versus Papular Speckles

Phacomatosis Pigmentokeratotica

Speckled lentiginous nevus: sometimes, but not always, part of a syndrome

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