Phacomatosis Pigmentokeratotica

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

doi:10.1001/archderm.134.3.333

Cited by 76 publications

(27 citation statements)

References 17 publications

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“…Ipsilateral hemi-atrophy, found in this case, was consistent with cases published in the literature, which was the most consistent extra-cutaneous anomaly in patients who had Phacomatosis pigmentokeratotica, which was one of the ENS in case series study conducted in 1998 (Tadini et al 1998). However, no previous reports for a patient with ENS who had short middle finger similar to this case.…”

Section: Discussion:-supporting

confidence: 90%

Hypophosphatemic Rickets, Epidermal Nevus Syndrome With Skeletal Changes: A Case Report.

Alzarnougi¹,

Agha²

2017

IJAR

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Section: Discussion:-supporting

confidence: 90%

Hypophosphatemic Rickets, Epidermal Nevus Syndrome With Skeletal Changes: A Case Report.

Alzarnougi¹,

Agha²

2017

IJAR

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“…On the other hand, malignant degeneration seems to occur less frequently when compared to macular SLN. There are only 6 reports of malignant melanoma originating from papular SLN [1, 16, 29,64,65,66]. Four of these patients were women.…”

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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“…On the skin, it is manifested as a combination of the linear nevus sebaceous and the speckled lentiginous nevus, also known as nevus spilus. Other associated features reported in patients with phacomatosis pigmentokeratotica most commonly included skeletal or neurological abnormalities 2, 3.…”

Section: Introductionmentioning

confidence: 99%