J Cutan Pathol 2017 Jul 14. Epub 2017 Jul 14.

Department of Dermpath Diagnostics, New York, Port Chester, NY, USA.

Background: We previously demonstrated the presence of onychodermis, a specialized mesenchymal cell population beneath the the nail matrix and proximal nail bed demonstrating CD10 expression. We hypothesize that the onychodermis could be the nail analog of the follicular dermal papilla, which is known to express CD13. We compare CD13 expression patterns between specialized mesenchymes of nail and hair, and compare these findings with CD10 expression patterns. Read More

Australas J Dermatol 2017 Jul 11. Epub 2017 Jul 11.

Dermatology and Skin Cancer Unit, Arcispedale S. Maria Nuova, Institute for Research and Health Care, Reggio Emilia, Italy.

Syringocystadenoma papilliferum is a benign adnexal neoplasm frequently seen in association with other adnexal tumours. We report the dermoscopic features of three cases of syringocystadenoma papilliferum developing in naevus sebaceus. Clinically the lesions were characterised by exophytic papillary structures. Read More

Skin Appendage Disord 2017 May 16;3(2):83-91. Epub 2017 Mar 16.

Department of Dermatology, University Hospital Hassan II, Fez, Morocco.

Background: Nevus sebaceus of Jadassohn (NSJ) is a complex cutaneous hamartoma with various clinical appearances.

Aim: To describe different dermoscopic patterns of this nevus according to its evolutionary stages.

Methods: This was an analytical study of NSJ carried out in the Department of Dermatology at the University Hospital Hassan II, Fez, Morocco. Read More

Pediatr Dermatol 2017 May;34(3):359-360

J Nippon Med Sch 2017 ;84(2):79-82

Department of Dermatology, Nippon Medical School.

We report a case of syringocystadenoma papilliferum (SCAP) combined with tubular papillary adenoma (TPA) arising on the chest of a 45-year-old Japanese woman. Histopathological examination revealed the characteristic findings of SCAP in the superficial part of the lesion and those of TPA in the deeper part. We reviewed the English literature about this combination. Read More

Surg Pathol Clin 2017 Jun;10(2):367-382

Department of Dermatology, University of Heidelberg, Im Neuenheimer Feld 440, 69120 Heidelberg, Germany; Labor für Dermatohistologie und Oralpathologie, Bayerstrasse 69, 80335 München, Munich, Germany. Electronic address:

Sebaceous skin tumors are classified into sebaceous adenoma, sebaceoma, and sebaceous carcinoma. An additional group of cystic sebaceous tumors indicate the Muir-Torre syndrome (MTS). Cystic sebaceous tumors are considered as morphologic variants of the 3 main categories. Read More

J Cutan Pathol 2017 Apr 17. Epub 2017 Apr 17.

Department of Pathology, Dermatopathology Division, Sylvester Comprehensive Cancer Center and Miller School of Medicine, University of Miami, Miami, Florida.

Nevus sebaceus of Jadassohn, a congenital cutaneous hamartoma, has the potential to develop into various epidermal adnexal-origin neoplasms. While the most common neoplasms are trichoblastoma or syringocystadenoma, proliferating trichilemmal cysts are exceptionally rare. We report a case of a 63-year-old Cuban male with a giant proliferating trichilemmal cyst arising from a nevus sebaceus on the right shoulder which had been growing for 30 years. Read More

Dermatol Surg 2017 Feb 17. Epub 2017 Feb 17.

Department of Dermatology, Houston Methodist Hospital, Houston, Texas The Surgery Center at Plano Dermatology, Plano, Texas Department of Dermatology, Kelsey-Seybold Clinic, Houston, Texas Department of Dermatology, University of Texas Southwestern Medical Center, Dallas, Texas.

J Cutan Pathol 2017 Jan 21;44(1):93-97. Epub 2016 Oct 21.

Department of Dermatology, Tufts Medical Center, Boston, MA, USA.

Trichilemmomas are benign cutaneous proliferations derived from the outer root sheath of the hair follicle. They most often occur on the head and neck region and show a female predominance. When multiple, they are associated with Cowden syndrome (CS), a rare disorder due to an autosomal dominant germline mutation in PTEN (phosphatase and tensin homolog on chromosome 10), a tumor suppressor gene. Read More

J Cutan Pathol 2016 Nov 1;43(11):1079-1080. Epub 2016 Sep 1.

Dermatopathology Friedrichshafen, Friedrichshafen, Germany.

Am J Dermatopathol 2016 Oct;38(10):762-5

*Department of Dermatology, The Third Xiangya Hospital of Central South University, Changsha, China; †Department of Surgery, and ‡Department of Pathology and Laboratory Medicine and Department of Dermatology, Hofstra Northwell School of Medicine, NY.

Syringocystadenocarcinoma papilliferum in situ, a variant of cutaneous adenocarcinoma in situ, is extremely rare. Only 9 cases have been published to date with 2 cases demonstrating pagetoid epidermal involvement. In this study, we report a case of syringocystadenocarcinoma papilliferum in situ with pagetoid epidermal involvement arising from a long-standing nevus sebaceus on the scalp of a 60-year-old woman. Read More

J Biol Regul Homeost Agents 2016 Apr-Jun;30(2 Suppl 2):17-20

Department of Medical 1Department of Pathology, Division of Dermatopathology, University of Virginia Health System, Charlottesville, Virginia.

Trichilemmoma and trichoblastoma are benign adnexal neoplasms derived from the hair follicle unit. While trichilemmomas are closely associated with the epidermis, trichoblastomas are found within the dermis and subcutaneous tissue. Both tumors have been reported to arise within nevus sebaceus of Jadassohn (NSJ). Read More

Case Rep Oncol 2016 Jan-Apr;9(1):143-7. Epub 2016 Feb 20.

Department of Dermatology, Hokkaido University Graduate School of Medicine, Sapporo, Japan.

Nevus sebaceus (NS) is a common congenital birthmark, and various tumors have been reported to develop in NS. Basal cell carcinoma (BCC) seldom occurs in NS, and it is very important to be able to clinicopathologically distinguish BCC from trichoblastoma. Herein, we describe a case of BCC and trichoblastoma occurring simultaneously in the same NS, including the differential dermoscopic features. Read More

J Dermatol 2016 Oct;43(10):1236-1237

Department of Dermatology, Saitama Medical University Saitama Medical Center, Saitama, Japan.

Dermatol Online J 2016 Nov 15;22(11). Epub 2016 Nov 15.

Specialist of Dermatology and Venereology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey.

Syringocystadenoma papilliferum is a rare cutaneous adnexal tumor that usually arises in the head and neck region. It may develop de novo or within a nevus sebaceus. Linear syringocystadenoma papilliferum is an uncommon variant of this benign tumor. Read More

Indian J Dermatol 2015 Nov-Dec;60(6):637

Department of Pathology, Hind Institute of Medical Sciences, Safedabad, Barabanki, Uttar Pradesh, India.

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprising of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual to develop two different types of tumor, benign and malignant, to arise simultaneously within a single sebaceus nevus. Read More

Handb Clin Neurol 2015 ;132:291-316

Departments of Dermatology and Community and Family Medicine, University of California San Francisco, Santa Rosa, CA, USA. Electronic address:

The term epidermal nevus syndrome (ENS) has been used to describe the association of epidermal hamartomas and extracutaneous abnormalities. Although many continue to use the term "ENS," it is now understood that this is not one disease, but rather a heterogeneous group with distinct genetic profiles defined by a common cutaneous phenotype: the presence of epidermal and adnexal hamartomas that are associated with other organ system involvement. One commonality is that epidermal nevi often follow the lines of Blaschko and it appears the more widespread the cutaneous manifestations, the greater the risk for extracutaneous manifestations. Read More

Indian J Pathol Microbiol 2015 Oct-Dec;58(4):534-6

Department of Pathology, Gülhane Military Medical Academy, Ankara, Turkey.

Nevus sebaceus (NS) is a congenital, benign, hamartomatous lesion and it is possible to see several benign or malignant tumors accompanying it. One of these is the poroma, which is very rare, and has only been reported twice before, in the English literature. In this paper, we presented two new cases of NS. Read More

Australas J Dermatol 2017 Feb 23;58(1):58-60. Epub 2015 Sep 23.

St John's Institute of Dermatology, King's College London, Guy's Hospital, London, UK.

Naevus sebaceus has recently been shown to result from post-zygotic mutations in HRAS, KRAS or occasionally NRAS. We present details of a neonate with extensive naevus sebaceus in whom we identified a pathogenic mutation in HRAS (c.37G > C; p. Read More

J Cutan Pathol 2015 Sep 22. Epub 2015 Sep 22.

Department of Cellular Pathology, Hospital El Bierzo, Ponferrada, Spain.

Int J Dermatol 2016 Feb 17;55(2):193-200. Epub 2015 Sep 17.

Department of Dermatology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Nevus sebaceus is a hamartoma composed of abnormal epidermal and dermal components with clinical and histopathological features that change with aging. Prophylactic excision of the lesion was advised historically considering its potential for giving rise to malignant tumors. However, whether early prophylactic excision should be performed has been questioned recently as most of the tumors have been found to be benign in origin. Read More

J Dermatol 2016 Feb 12;43(2):175-80. Epub 2015 Sep 12.

Department of Dermatology, National Taiwan University Hospital and College of Medicine, Taipei, Taiwan.

Nevus sebaceus is frequently associated with the development of secondary neoplasms. Incidences of malignant transformation vary among different reports and few data is available regarding Asian populations. We aimed to determine the characteristics of secondary tumors developing from nevus sebaceus in a Taiwanese population and to review the published work. Read More

Am J Dermatopathol 2016 Jan;38(1):56-62

Departments of *Pathology, and †Dermatology, University of Alabama at Birmingham, Birmingham, AL.

Syringocystadenocarcinoma papilliferum (SCACP) is an extremely rare adnexal neoplasm, believed to arise in a preexisting nevus sebaceus of Jadassohn (NSJ) through a multistep progression process. This hypothetical process involves an NSJ giving rise to syringocystadenoma papilliferum, which then presumably undergoes malignant transformation in rare circumstances to give rise to SCACP in situ, which finally progresses to an invasive SCACP. Of the 30 SCACP cases reported so far, none have documented the process from a birthmark to the final invasive lesion, with histological evidence of each step, in a single tumor. Read More

Int J Pediatr Otorhinolaryngol 2015 Nov 20;79(11):1932-4. Epub 2015 Jul 20.

Medical University of Warsaw, Department of Pediatric Otolaryngology, Poland.

Nevus sebaceus of Jadassohn is a congenital yellowish hairless skin lesion, mainly located on the head and neck. A common phenomenon is the coexistence of secondary tumors within the lesion. These are mainly benign tumors, the majority of which are trichoblastoma and syringocystadenoma papilliferum. Read More

Eplasty 2015 20;15:ic38. Epub 2015 Jul 20.

Division of Plastic Surgery, Department of Surgery, Morsani College of Medicine, University of South Florida, Tampa.

Australas J Dermatol 2015 May;56(2):e53-4

Department of Dermatology, Faculty of Medicine, Hacettepe University, Ankara, Turkey.

Am J Clin Dermatol 2015 Jun;16(3):197-204

Albert Einstein College of Medicine, Bronx, NY, USA.

Nevus sebaceus (NS) and Becker's nevus (BN) are two variants of epidermal nevi. NS clinically presents as a yellowish-orange, hairless plaque on the scalp, face, or neck, while BN presents as a tan-to-brown hyperpigmented, sometimes hypertrichotic, plaque typically on the chest and shoulder. Histologically, NS displays mature or nearly mature sebaceus glands as well as acanthosis and fibroplasia of the papillary dermis. Read More

J Cutan Pathol 2015 Sep 12;42(9):645-51. Epub 2015 May 12.

Department of Dermatology, University of Iowa, Iowa City, IA, USA.

Herein, we describe a 63-year-old male with multiple tumors arising within a nevus sebaceus on the posterior scalp. On histopathologic examination, four distinct tumors were identified: trichoblastoma, syringocystadenoma papilliferum, desmoplastic trichilemmoma and tumor of the follicular infundibulum (TFI). Within the TFI component of the nevus sebaceus, there was intracytoplasmic accumulation of eosinophilic keratin, as shown on pancytokeratin-stained sections, imparting a signet-ring appearance to the cells. Read More

Ann Lab Med 2015 Jan 8;35(1):178-80. Epub 2014 Dec 8.

Department of Pediatrics, Busan Paik Hospital, Inje University College of Medicine, Busan, Korea.

J Cutan Pathol 2014 Dec 11;41(12):944-9. Epub 2014 Nov 11.

Department of Dermatology, Hospital Infanta Cristina, Madrid, Spain.

Ductal carcinoma in situ (DCIS) in the mammary glands consists of a proliferation of ductal luminal cells with preserved polarity but lacking the marked intercellular cohesion of hyperplastic cells. We report two cases of DCIS arising in the apocrine glandular structures of a nevus sebaceus (NS). The first patient was a 44-year-old woman with a ductal neoplasm composed of multilayered ductal elements with a monomorphous cellularity and a polar orientation of nuclei. Read More

Hautarzt 2014 Dec;65(12):1022-9

Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universitätsklinikum Würzburg, Josef-Schneider-Straße 2, 97080, Würzburg, Deutschland.

Unusual congenital or early-onset skin lesions on the scalp often pose a diagnostic challenge particularly as the clinical evaluation may be hampered by dense hair growth. Thus, this paper provides a concise review on developmental abnormalities and nevi with exclusive or predominant scalp localization. Aplasia cutis congenita occurs as an isolated finding, in association with genetic syndromes, nevi and anomalies or as a consequence of intrauterine trauma and teratogens. Read More

Ann Dermatol 2014 Aug 31;26(4):501-4. Epub 2014 Jul 31.

Department of Dermatology, Kyungpook National University School of Medicine, Daegu, Korea.

Epidermal nevi (EN) are hamartomatous lesions derived from epidermal components originating from pluripotent cell mutations. They have been categorized according to their predominant component. The existence of >2 types of EN concurrently within a single area or within contiguous areas has been rarely reported. Read More

Dermatol Online J 2014 Jul 15;20(7). Epub 2014 Jul 15.

School of Medicine Temple University.

A 14-year-old girl presented with a new, rapidly growing, pigmented tumor on a previously existing yellowish, verrucous plaque on the scalp. The patient received complete surgical excision. Routine histology ruled out basal cell carcinoma (BCC) and the histological diagnosis was pigmented trichoblastoma arising in nevus sebaceous (NS). Read More

J Dermatol Case Rep 2014 Jun 30;8(2):58-9. Epub 2014 Jun 30.

Department of Dermatology, Freiburg University Medical Center, Freiburg, Germany.

Phacomatosis pigmentokeratotica is characterized by the coexistence of nevus sebaceus, papular nevus spilus and associated neurologic abnormalities. We report a case of phacomatosis pigmentokeratotica in a 28-year-old male who presented with palmar-plantar dysesthesia and ipsilateral brain hemiatrophy. As a characteristic neuroimaging finding of the disorder, we found multiple hypointense lesions involving the ipsilateral hemisphere. Read More

Curr Opin Pediatr 2014 Aug;26(4):452-9

Department of Dermatology and Pediatrics, Oregon Health and Science University, Portland, Oregon, USA.

Purpose Of Review: Children and their parents typically present to pediatricians to discuss treatment options for melanocytic nevi, nevus sebaceus, port-wine stains, and hemangiomas. Each of these conditions may be medically managed, but in some situations surgical intervention may be preferable.

Recent Findings: Although recent studies have improved our understanding of melanoma risk among patients with congenital nevi, prospective trials are needed to more accurately assess whether surgical excision mitigates this risk. Read More

Br J Dermatol 2014 Nov 30;171(5):1073-7. Epub 2014 Sep 30.

Department of Pathology, National Taiwan University Hospital, Taipei, Taiwan; Graduate Institute of Pathology, College of Medicine, National Taiwan University, Taipei, Taiwan.

Background: Trichilemmoma is a benign follicular epithelial tumour exhibiting outer root sheath differentiation. It is associated with Cowden syndrome and naevus sebaceus (NS), but the pathogenesis of sporadic tumours is poorly understood. Recently, NS was found to be caused by postzygotic HRAS or KRAS mutations. Read More

Dermatol Online J 2014 Apr 16;20(4):22335. Epub 2014 Apr 16.

United States Navy.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare malignant counterpart of syringocystadenoma papilliferum (SCAP). To date, less than 30 cases have been reported, and of those, only three had locoregional metastases [1,2]. Herein, we report a case of a 42-year-old man with a rapidly growing nodule on his right parietal scalp ultimately diagnosed as SCACP arising in association with a preexisting SCAP and nevus sebaceus. Read More

J Cutan Pathol 2014 Jun 2;41(6):509-12. Epub 2014 Apr 2.

Department of Pathology, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Tao-Yuan, Taiwan.

Nevus sebaceus is known to have the potential to develop into various secondary tumors. We observed a sebaceoma arising from a nevus sebaceus excised from the left cheek of a 51-year-old woman. This sebaceoma showed desmoplastic change similar to that observed in desmoplastic trichoepithelioma and desmoplastic trichilemmoma. Read More

Int J Health Sci (Qassim) 2013 Nov;7(3):343-6

Assistant Professor and Consultant of Histopathology and Dermatopathology, King Khalid University hospital, King Saud University, P.O. Box 2925, Riyadh - 11461, Department of Pathology (32), Riyadh, Kingdom of Saudi Arabia.

Although nevus sebaceous (NS) is known to develop various types of secondary neoplasms, it rarely causes carcinoma and only fifteen cases of secondary squamous cell carcinoma (SCC) have been reported. We report a rare variant of SCC (acantholytic type) arising in nevus sebaceus. Read More

Korean J Pathol 2013 Dec 24;47(6):569-74. Epub 2013 Dec 24.

Department of Family Medicine, Diskapi Yildirim Beyazit Training and Research Hospital, Ankara, Turkey. ; Department of Family Medicine, University of Antwerp, Antwerp, Belgium.

Nevus sebaceus of Jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. While multiple tumors may occasionally arise, it is unusual for more than four tumors to arise simultaneously within a single sebaceus nevus. Read More

Dermatol Online J 2013 Dec 16;19(12):20722. Epub 2013 Dec 16.

New York University School of Medicine.

We present a case of a 35-year-old woman with a yellow, verrucous, and itchy plaque on her scalp. Within this plaque, there was an erythematous, bleeding papule. Histopathologic findings were compatible with a diagnosis of syringocystadenoma papilliferum within a nevus sebaceous. Read More

Clin Exp Dermatol 2014 Jan;39(1):1-6

Dermatology Centre, Salford Royal NHS Foundation Trust, Stott Lane, University of Manchester, Manchester, UK.

Epidermal naevi are common cutaneous mosaic disorders that occur in 0.1-0.3% of live births. Read More

Eur J Dermatol 2014 Jan-Feb;24(1):70-5

Department of Dermatology and Allergy, Technische Universität München, Biedersteiner Strasse 29, 80802 Munich, Germany.

Background: The preferential occurrence of certain skin neoplasms on the scalp of children raises concerns from their parents and warrants special diagnostic and therapeutic approaches.

Objective: To explore the demographic and clinical characteristics of scalp neoplasms in the pediatric population, with attention to malignant tumors and systemic syndromes.

Methods: Scalp neoplasms in patients aged 12 years or younger were retrospectively collected in 1990-2010 from two tertiary referral centers in Taiwan. Read More

Pediatr Dermatol 2014 Mar-Apr;31(2):208-11. Epub 2013 Dec 13.

Division of Dermatology, University of Louisville, Louisville, Kentucky.

Meningothelial hamartoma is a rare lesion of ectopic meningothelial elements that usually presents on the scalp at birth or in early childhood. The characteristic spindle cells infiltrate through collagen and stain positively for epithelial membrane antigen and vimentin but are negative for CD31 and CD34. This distinguishes meningothelial hamartoma from vascular tumors. Read More

J Am Acad Dermatol 2014 Feb 20;70(2):332-7. Epub 2013 Nov 20.

Ackerman Academy of Dermatopathology, New York, New York.

Background: Nevus sebaceus is commonly associated with the development of secondary neoplasms. Data on the frequency of malignant tumors vary considerably in published reports.

Objective: We sought to analyze the distribution of secondary neoplasm in nevus sebaceus. Read More

Pediatr Dermatol 2014 May-Jun;31(3):326-30. Epub 2013 Nov 14.

Department of Dermatology, Stanford University School of Medicine, Stanford, California.

Nevus sebaceus (NS) is a common congenital cutaneous hamartoma that typically presents on the scalp and face at birth or in early childhood. Occasionally NS can be associated with the Schimmelpenning-Feuerstein-Mims syndrome, which presents with concomitant severe neurologic, skeletal, cardiovascular, ophthalmic, and genitourologic disorders. In a previous study, maternal transmission of the human papillomavirus (HPV) and infection of ectodermal stem cells by HPV was postulated to result in the development of NS. Read More

J Invest Dermatol 2013 Aug;133(8):1923-5

Department of Dermatology, Freiburg University Medical Center, University of Freiburg, Freiburg, Germany.

In phacomatosis pigmentokeratotica, papular nevus spilus coexists with nevus sebaceus. The disorder was thought to be a didymosis with early postzygotic recombination. In this issue, however, Groesser and co-workers provide a new concept. Read More

J Cutan Pathol 2013 Jul 2;40(7):676-82. Epub 2013 Apr 2.

National Skin Centre, Singapore, Singapore.

We report a case of a 48-year-old Malay female who presented with multiple tumors arising from a large nevus sebaceus on her right parieto-temporal scalp. Histologically, the tumors corresponded to a sebaceoma with carcinomatous change, a poroma and a trichoblastoma. Immunohistochemical staining of the sebaceous tumor with p53 showed strong within the areas of carcinomatous change, while there was negative to weak staining within the sebaceoma-like areas. Read More

J Cutan Pathol 2013 May 14;40(5):455-62. Epub 2013 Mar 14.

Department of Dermatology, Cleveland Clinic Foundation, Cleveland, OH, USA.

Background: Until the 1990s, basal cell carcinoma (BCC) was viewed as the most common epithelial neoplasm developing in association with nevus sebaceus (NS). Currently, trichoblastoma is thought of as the most prevalent basaloid neoplasm in NS. The follicular stem cell marker pleckstrin homology-like domain, family A, member 1 (PHLDA1) also known as T-cell death-associated gene 51 (TDAG51) labels trichoepithelioma (TE) but not BCC. Read More

J Invest Dermatol 2013 Mar;133(3):597-600

Department of Dermatology, Freiburg University Medical Center, University of Freiburg, Freiburg, Germany.

The recent discovery that nevus sebaceus is a mosaic RASopathy represents a major breakthrough in research on epidermal nevi. In this issue, both Levinsohn et al. and Sun et al. Read More