Familial papular epidermal nevus with “skyline” basal cell layer and multiple pilomatricomas: A new association?

Rizzoli, Laura; Balestri, Riccardo; Rech, Giulia; Zorzi, Maria Grazia; Speziali, Luigi; Pedrolli, Annalisa; Girardelli, Carlo Renè

doi:10.1111/pde.13428

Cited by 4 publications

(5 citation statements)

References 14 publications

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“…Cutaneous associations such as pilomatricomas have been reported in PENS, 12 whereas malignant transformation of PENS or association with other malignancies has not been reported. 10–12…”

Section: Commentmentioning

confidence: 99%

“…further 5 cases having developed PENS lesions within their first year of life. [2][3][4][5][6][7][8][9][10][11][12][13] In 2 cases, the exact age of development had not been reported. 6,10,11 New lesions can develop within months or up to 3 years after onset, and the total number may go up to as high as 18.…”

mentioning

confidence: 99%

“…5 PENS with neurological manifestations has been termed PENS syndrome. 12 Reported neurological symptoms generally appear during childhood, and include febrile seizures, epilepsy, speech delay, and psychomotor retardation. These appear to be self-limiting with a favorable prognosis.…”

mentioning

confidence: 99%

“…13 It has been postulated that individuals with more than 4 PENS lesions have a relative risk of 6.3 times for developing neurological manifestations, 7 with a recommendation to refer these patients to a paediatric neurologist to exclude neurologic delays. 7 Cutaneous associations such as pilomatricomas have been reported in PENS, 12 whereas malignant transformation of PENS or association with other malignancies has not been reported. [10][11][12] In conclusion, PENS is a rare subtype of epidermal nevi that is not commonly recognized and under diagnosed.…”

mentioning

confidence: 99%

“…7 Cutaneous associations such as pilomatricomas have been reported in PENS, 12 whereas malignant transformation of PENS or association with other malignancies has not been reported. [10][11][12] In conclusion, PENS is a rare subtype of epidermal nevi that is not commonly recognized and under diagnosed. Because of its associations with neurological disorders, dermatologists and dermatopathologists need to have a high index of suspicion for early accurate diagnosis and timely interventions.…”

mentioning

confidence: 99%

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Papular epidermal nevus with "skyline" basal cell layer (PENS). COMMENTEpidermal nevi are benign overgrowths of epidermal keratinocytes. They typically present at birth or in infancy as a linear hyperpigmented patch or plaque, following lines of Blaschko. With time, lesions may become thicker and more verrucous. 1 Although most epidermal nevi are isolated to the skin, rare cases of extensive epidermal nevi may be associated with abnormalities of the eyes, skeletal, cardiovascular, urogenital, or central nervous system. 1 PENS is a rare subtype of epidermal nevi. Interestingly, 50% of reported cases of PENS have been associated with neurological abnormalities in childhood. 2 The syndrome was first described by Caputo and Tadini in 2006, and in 2011, the term PENS was coined. 3,4 PENS most commonly presents as asymptomatic, small, round or polygonal hyperkeratotic papules or plaques. The color of lesions can range from white to brown, [4][5][6]10,13 and in some cases, lesions of different color may be present in the same individual. 7,10,11 Unlike epidermal nevi that occur linearly along lines of Blaschko, 4 PENS lesions do not typically follow a similar distribution and mostly occur in a sporadic distribution, with a predilection for the face, neck, trunk, and extremities, while sparing the palms and soles. 4 Only 2 PENS cases have been described following a Blaschkoid distribution. 6,7 To date, 22 cases of PENS have been reported in the literature. In 15 cases, lesions were present at birth, with a From the

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“…Cutaneous associations such as pilomatricomas have been reported in PENS, 12 whereas malignant transformation of PENS or association with other malignancies has not been reported. 10–12…”

Section: Commentmentioning

confidence: 99%