Cerebriform Nevus Sebaceous in a Neonate
T
he first clinical examination of a term Nigerian newborn 3 hours after vaginal delivery showed a salmon red, convoluted lesion on the scalp (Figure 1, A). The lesion was not painful, dry, and gummy to touch; no hair grew in the area of interest. The lesion’s appearance was consistent with that of cerebriform nevus sebaceous (NS), a quite rare variant of the more common “classic” NS. Pregnancy and delivery were uneventful; maternal serologies and vaginal swab were negative. No other skin lesions were detected. Remaining clinical examination was unremarkable. As a diagnostic completion, a cerebral echography was performed, turning out normal. The scalp lesion was reevaluated 3 days later before discharge, appearing paler and shrinking (Figure 1, B). One month later, we reevaluated the baby: the scalp lesion grew paler further and lost the initial swollen appearance, taking the mature aspect of a NS (Figure 2). The rest of the clinical examination at 30 days of life (including neurological examination) was normal. NS is a hamartomatous composite lesion recognizing epidermal, sebaceous, apocrine, and immature hair follicular elements. Although ultimate validation may be obtained with histology, diagnosis is usually made clinically thanks to NS typical appearance (congenital verrucous, granulated, yelloworange plaque that may be round, crescent, or linear in shape), and position (most frequently on the scalp, 59.3%; face, 32.6%; preauricular area, 3.8%; neck, 3.2%; locations off the head and neck, 1.3%).1 Natural history of NS classically passes through 3 overlapping phases. The first (infantile) stage is characterized by plain nevus papillomatous hyperplasia and immature hair follicles; the second stage starts with puberty, when a rapid growth of the nevus (its surface shifting from plain to verrucous) occurs because of hormone-driven development of sebaceous
elements and maturation of apocrine glands; the third stage consists of onset of benign (16% of cases) and malignant (8% of cases) epithelial neoplasms in the context of NS.2 Although there is wide consensus on extensive excision of NS in case of malignant neoplasms development, there is no complete agreement about the opportunity to perform prophylactic surgical excision vs close follow-up of lesions in first and second stages.2 Widespread cutaneous NS (particularly those spreading over 2 or more dermatomes) may be part of a complex syndrome, known as linear nevus sebaceous syndrome: this may encompass skeletal, ocular, and neurological abnormalities, plus various other possible disorders (eg, patent ductus arteriosus, aortic coarctation, horse-shoe kidney, undescended testes, hepatosplenomegaly, and failure to thrive).3 Cerebriform NS is an atypical, rare variant of NS, first described in a 20-year-old man by Ramesh et al in 1998.4 Subsequent reports included Bomsztyk et al in a 6-month-old child,5 and by Correale et al in 5 neonates.6 Causative factors of the cerebriform appearance are still not clearly understood, and prognosis is comparable with that of classical isolated NS. n Enrico Valerio, MD Veronica Mardegan, MD Cristina Zanella, MD Paola Gaio, MD Department of Woman and Child Health Medical School University of Padua Padova, Italy
Mario Cutrone, MD Department of Pediatrics Ospedale Dell’Angelo, Mestre (VE), Italy
Figure 1. A, Cerebriform NS 3 hours after birth. B, Same lesion 72 hours after birth, appearing paler and shrinking.
J Pediatr 2014;164:665-6. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.10.028
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THE JOURNAL OF PEDIATRICS
www.jpeds.com
Vol. 164, No. 3
References 1. Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes: part I. J Am Acad Dermatol 2009;61:549-60. 2. Moody MN, Landau JM, Goldberg LH. Nevus sebaceous revisited. Pediatr Dermatol 2012;29:15-23. 3. Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes: part II. J Am Acad Dermatol 2009;61:563-78. 4. Ramesh A, Murugusundaram S, Vittel K, Kumar S, Janaki VR, Boopalraj JM. Cerebriform sebaceous nevus. Int J Dermatol 1998;37:220. 5. Bomsztyk ED, Garzon MC, Ascherman JA. Postauricular cerebriform sebaceous nevus: case report and literature review. Ann Plast Surg 2008; 61:637-9. 6. Correale D, Ringpfeil F, Rogers M. Large, papillomatous, pedunculated nevus sebaceus: a new phenotype. Pediatr Dermatol 2008;25:355-8.
Figure 2. Cerebriform NS at 30 days of life, showing the classic NS appearance.
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Valerio et al
T
he first clinical examination of a term Nigerian newborn 3 hours after vaginal delivery showed a salmon red, convoluted lesion on the scalp (Figure 1, A). The lesion was not painful, dry, and gummy to touch; no hair grew in the area of interest. The lesion’s appearance was consistent with that of cerebriform nevus sebaceous (NS), a quite rare variant of the more common “classic” NS. Pregnancy and delivery were uneventful; maternal serologies and vaginal swab were negative. No other skin lesions were detected. Remaining clinical examination was unremarkable. As a diagnostic completion, a cerebral echography was performed, turning out normal. The scalp lesion was reevaluated 3 days later before discharge, appearing paler and shrinking (Figure 1, B). One month later, we reevaluated the baby: the scalp lesion grew paler further and lost the initial swollen appearance, taking the mature aspect of a NS (Figure 2). The rest of the clinical examination at 30 days of life (including neurological examination) was normal. NS is a hamartomatous composite lesion recognizing epidermal, sebaceous, apocrine, and immature hair follicular elements. Although ultimate validation may be obtained with histology, diagnosis is usually made clinically thanks to NS typical appearance (congenital verrucous, granulated, yelloworange plaque that may be round, crescent, or linear in shape), and position (most frequently on the scalp, 59.3%; face, 32.6%; preauricular area, 3.8%; neck, 3.2%; locations off the head and neck, 1.3%).1 Natural history of NS classically passes through 3 overlapping phases. The first (infantile) stage is characterized by plain nevus papillomatous hyperplasia and immature hair follicles; the second stage starts with puberty, when a rapid growth of the nevus (its surface shifting from plain to verrucous) occurs because of hormone-driven development of sebaceous
elements and maturation of apocrine glands; the third stage consists of onset of benign (16% of cases) and malignant (8% of cases) epithelial neoplasms in the context of NS.2 Although there is wide consensus on extensive excision of NS in case of malignant neoplasms development, there is no complete agreement about the opportunity to perform prophylactic surgical excision vs close follow-up of lesions in first and second stages.2 Widespread cutaneous NS (particularly those spreading over 2 or more dermatomes) may be part of a complex syndrome, known as linear nevus sebaceous syndrome: this may encompass skeletal, ocular, and neurological abnormalities, plus various other possible disorders (eg, patent ductus arteriosus, aortic coarctation, horse-shoe kidney, undescended testes, hepatosplenomegaly, and failure to thrive).3 Cerebriform NS is an atypical, rare variant of NS, first described in a 20-year-old man by Ramesh et al in 1998.4 Subsequent reports included Bomsztyk et al in a 6-month-old child,5 and by Correale et al in 5 neonates.6 Causative factors of the cerebriform appearance are still not clearly understood, and prognosis is comparable with that of classical isolated NS. n Enrico Valerio, MD Veronica Mardegan, MD Cristina Zanella, MD Paola Gaio, MD Department of Woman and Child Health Medical School University of Padua Padova, Italy
Mario Cutrone, MD Department of Pediatrics Ospedale Dell’Angelo, Mestre (VE), Italy
Figure 1. A, Cerebriform NS 3 hours after birth. B, Same lesion 72 hours after birth, appearing paler and shrinking.
J Pediatr 2014;164:665-6. 0022-3476/$ - see front matter. Copyright ª 2014 Mosby Inc. All rights reserved. http://dx.doi.org/10.1016/j.jpeds.2013.10.028
665
THE JOURNAL OF PEDIATRICS
www.jpeds.com
Vol. 164, No. 3
References 1. Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes: part I. J Am Acad Dermatol 2009;61:549-60. 2. Moody MN, Landau JM, Goldberg LH. Nevus sebaceous revisited. Pediatr Dermatol 2012;29:15-23. 3. Eisen DB, Michael DJ. Sebaceous lesions and their associated syndromes: part II. J Am Acad Dermatol 2009;61:563-78. 4. Ramesh A, Murugusundaram S, Vittel K, Kumar S, Janaki VR, Boopalraj JM. Cerebriform sebaceous nevus. Int J Dermatol 1998;37:220. 5. Bomsztyk ED, Garzon MC, Ascherman JA. Postauricular cerebriform sebaceous nevus: case report and literature review. Ann Plast Surg 2008; 61:637-9. 6. Correale D, Ringpfeil F, Rogers M. Large, papillomatous, pedunculated nevus sebaceus: a new phenotype. Pediatr Dermatol 2008;25:355-8.
Figure 2. Cerebriform NS at 30 days of life, showing the classic NS appearance.
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Valerio et al
