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Dermatol Surg 2019 Feb 7. Epub 2019 Feb 7.

Dermatology and Laser Surgery Center, Houston, Texas West Lake Dermatology, Austin, Texas Dermatology and Laser Surgery Center, Houston, Texas.

Semin Diagn Pathol 2019 Jan 11;36(1):48-61. Epub 2018 Dec 11.

Department of Pathology and Cell Biology - USF Health, Ruffolo, Hooper, and Associates, Tampa, FL, United States. Electronic address:

Hamartomas are benign lesions composed of aberrant disorganized growth of mature tissues. Choristomas are similar, except that they are composed of tissues not normally found at the anatomic site in which the lesion is arising. A wide range of hamartomas and choristomas can arise in the skin and soft tissue. Read More

Br J Dermatol 2018 Dec;179(6):1432-1433

Department of Dermatovenereology, West China Hospital, West China Hospital of Stomatology, Sichuan University, Chengdu, 610041, China.

Cutis 2018 Oct;102(4):291-294

Nova Southeastern University College of Osteopathic Medicine/Largo Medical Center, Florida, USA.

Nevus sebaceus (NS)(organoid nevus) is a benign follicular neoplasm that commonly occurs on the scalp, face, or neck. With time, it may give rise to benign or malignant tumors such as trichoblastoma, syringocystadenoma papilliferum, and basal cell carcinoma (BCC). Rarely, other types of neoplasms may develop. Read More

Am J Dermatopathol 2018 Oct 29. Epub 2018 Oct 29.

Sikl's Department of Pathology, Medical Faculty in Pilsen, Charles University in Prague, Pilsen, Czech Republic.

Syringocystadenoma papilliferum (SCAP) is a benign tumor most commonly located on the head and neck area often associated with nevus sebaceus. In its usual location, the human papillomavirus (HPV) DNA and mutations in the RAS/mitogen-activated protein kinase signaling pathway have been detected in SCAP. We studied 16 cases of SCAP in the anogenital areas and buttock where this neoplasm is rare and attempted to find out whether SCAP in these sites have different histopathological and molecular biological features. Read More

Pediatr Dermatol 2018 Nov;35(6):854-855

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

J Craniofac Surg 2019 Jan;30(1):184-185

Division of Plastic Surgery, Children's National Health System, Washington, DC.

Schimmelpenning syndrome is a neurocutaneous disorder characterized by craniofacial nevus sebaceus that fall along embryonic cutaneous lines and tend to be associated with neurological, ocular, skeletal, and vascular abnormalities. We report a child with extensive nevus sebaceus of the scalp, face, and thorax and other unusual physical findings who was found to have a mosaic mutation of KRAS c.35G>A p. Read More

Indian J Dermatol Venereol Leprol 2018 Jul 31. Epub 2018 Jul 31.

Department of Dermatology, Ramon y Cajal University Hospital, Madrid, Spain.

J Cutan Pathol 2018 Oct 3;45(10):777-781. Epub 2018 Aug 3.

Departamento de Morfología y Biología Celular, Grupo SINPOS, Universidad de Oviedo, Oviedo, Spain.

A vulvar case of nevus sebaceus is presented. During the routine histopatological examination, attention was drawn by several corpuscular structures. Immunohistochemistry demonstrated that they were sensory corpuscles, identified respectively as Meissner-like and glomerular corpuscles. Read More

Hum Pathol 2018 03 12;73:59-65. Epub 2017 Dec 12.

Department of Pathology, National Taiwan University Hospital and National Taiwan University College of Medicine, Taipei 10051, Taiwan.

Tubular apocrine adenoma (TAA) and papillary eccrine adenoma (PEA) are benign sweat gland tumors. Their names imply that they exhibit apocrine and eccrine differentiation, respectively. However, morphologically they are very similar and are often indistinguishable. Read More

Int J Dermatol 2018 01 31;57(1):120-122. Epub 2017 Oct 31.

Department of Pathology, Immunology, and Laboratory Medicine, University of Florida College of Medicine, Gainesville, FL, USA.

Pediatr Dermatol 2018 Jan 16;35(1):21-29. Epub 2017 Oct 16.

Department of Dermatology, University of California, San Francisco, Santa Rosa, CA, USA.

Knowledge of the molecular underpinnings of many epidermal nevi and epidermal nevus syndrome has expanded rapidly in recent years. In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus, inflammatory linear verrucous epidermal nevi, and cutaneous-skeletal hypophosphatemia syndrome. We will discuss how newly defined mutations relate to the biology reflected in the cutaneous patterns seen in these mosaic disorders and how new molecular data has informed our understanding of these diseases and shaped management decisions. Read More

Tunis Med 2016 Dec;94(12):898

Arch Craniofac Surg 2017 Mar 25;18(1):46-49. Epub 2017 Mar 25.

Department of Pathology, Busan-Baik Hospital, Inje University School of Medicine, Busan, Korea.

Nevus sebaceus is a hamartoma of the sebaceous gland that occurs congenitally, from which various secondary tumors can arise with a prevalence of 5%-6%. Benign neoplasms commonly arise from nevus sebaceous, but they have a very low malignant potential. Two neoplasms may occasionally arise within the same lesion, but it is rare for three or more neoplasms to occur in a nevus sebaceus simultaneously. Read More

J Clin Diagn Res 2017 Jul 1;11(7):MD09-MD10. Epub 2017 Jul 1.

Junior Resident, Department of Ear, Nose and Throat, Sri Siddhartha Medical College, Agalakote, Karnataka, India.

Nevus sebaceus of Jadassohn is a congenital tumour affecting the scalp and face. It is usually presented as a pigmented patch or plaque. It is a complex cutaneous hamartoma which involves pilosebaceous follicle, epidermis and adnexal structures. Read More

J Cutan Pathol 2017 Nov 18;44(11):909-914. Epub 2017 Aug 18.

Dermpath Diagnostics, Port Chester, New York.

Background: We previously demonstrated the presence of onychodermis, a specialized mesenchymal cell population beneath 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 2018 Feb 11;59(1):e59-e61. 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 09;43(9):1181-1184

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.

Pol J Pathol 2017;68(4):277-283

President of prewar Lvov and Polish Republic on Exile, associate professor Stanisław Ostrowski was a dermatologist with a keen interest in dermatopathology. This study was based on original resources, which - mainly reports of his own authorship - were focused on dermatopathology. Stanisław Ostrowski provided excellent description of naevus epitheliomatosus sebaceus Wolters-Friboes both in Polish and German to be cited after decades in renowned handbooks of dermatopahtology published by Springer Verlag. Read More

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 11 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 Dec 22;42(12):1047-1048. 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