We describe two sibs a boy and a girl suffering from linear nevus sebaceus of jadassohn. Nevus sebaceus of jadassohn nsj is a benign, congenital hamartoma that often presents at birth, appears to regress in childhood, and grows during puberty, suggesting possible hormonal. Nevus sebaceus of jadassohn is a congenital abnormality first described by the dermatologist josef jadassohn in 1895. Blue nevus of jadassohntiche also called common blue nevus is a very common type of blue nevus which i encounter frequently in both my the woodlands dermatology and conroe.
Examples of dermal nevi are the miescher nevus found on the face, and the unna nevus found on the body. Cerebriform nevus sebaceous of jadassohn a rare case report. Nevus sebaceous also known as nevus sebaceous of jadassohn ns is an epidermal nevi predominantly congenital sebaceous hamartoma. Jul 20, 2015 nevus sebaceus of jadassohn is a congenital abnormality first described by the dermatologist josef jadassohn in 1895. The nevus sebaceous of jadassohn is a little known entity to most otolaryngologists. They may be composed of a variety of epidermal cells. Pigmentary nevus, which may be congenital or generally of a circumscribed. Request pdf secondary neoplasms associated with nevus. Nevus sebaceous of jadassohn is a congenital sebaceous gland hamartoma usually found on the scalp and face. Epidermal nevi are benign, hamartomatous growths of the skin that are present at birth or develop in early childhood. Skin tumor nonmelanocytic nevus sebaceus of jadassohn.
Management of nevus sebaceous and the risk of basal cell. Nevus sebaceus of jadassohn nsj, or organoid nevus, is a complex cutaneous hamartoma, involving not only the pilosebaceous follicle, but also the epidermis and often other adnexal. Nevus sebaceous is a congenital hamartoma of the skin, defined. The authors should have referred to three fairly recent articles which would have clarified the diagnosis of the. Intraoral blue jadassohntieche nevus sciencedirect. Linear sebaceous nevus syndrome jama pediatrics jama network.
Pathology of nevus sebaceous organoid nevus dr sampurna. Well go over some of the most common types and how to recognize them. Dec 30, 2019 nevus sebaceus, also called nevus sebaceus of jadassohn or organoid nevus, is a benign hamartoma of the skin, characterized by hyperplasia of the epidermis, immature hair follicles, and sebaceous and apocrine glands. Additional terms used to describe nevus sebaceus syndrome include schimmelpenning syndrome, schimmelpenningfeuersteinmims syndrome, linear sebaceous nevus sequence, nevus sebaceous of jadassohn, jadassohn nevus phacomatosis and jadassohn sebaceous nevus syndrome. Malignant transformation of a papillary yellowish nevus sebaceus of jadassohn to basal cell carcinoma aggregated asymmetrical ovoid nets with. Large tan macule dotted with numerous superimposed small dark nevi. Pathology outlines nevus with features of congenital onset. Epidermal nevus syndromes nord national organization. Lesions are usually present at birth and appear as waxy, yelloworange or tan, hairless plaques. Nevus sebaceous of jadassohn jama otolaryngologyhead. This website is intended for pathologists and laboratory personnel, who understand that medical information.
Nevus sebaceous of jadassohn jama pediatrics jama network. It is composed of sebaceous glands and commonly presents as. Nevus or nevi if multiple is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. Nevus lipomatosus cutaneus superficialis associated with. Although access to this page is not restricted, the information found here is intended for use by medical providers. It has the potential to develop into variety of neoplasms of various epidermal. Patients should address specific medical concerns with their physicians. Even though present at birth, it is more readily seen at 1 month to 6 weeks of age. Pdf association of nevus of jadassohn, sebaceoma and. Nevus sebaceous of jadassohn is a common skin lesion of children. Key points epidermal nevus syndrome is an inclusive term for congenital disorders characterized by epidermal nevi as the.
Nevus sebaceus of jadassohn nsj is a benign, congenital hamartoma that often presents at birth, appears to regress in childhood, and. Nevus sebaceous of jadassohn definition circumscribed lesion that occurs mainly on the face and scalp and consists predominantly of sebaceous glands, abortive hair follicles and ectopic apocrine glands. Secondary neoplasms associated with nevus sebaceus of jadassohn. This means that nevus sebaceous of jadassohn, or a subtype of nevus sebaceous of jadassohn, affects less than 200,000 people in the us population. We report the case of a nevus sebaceous of jadassohn in its third stage in a 42yearold male patient. Association of nevus of jadassohn, sebaceoma and trichoblastoma in a scalp lesion. Nevus sebaceous of jadassohn symptoms, diagnosis, treatments. It presents as a group of verrucous, closely grouped, skincolored to brown papules often in a linear arrangement following the. A benign, exophytic, usually pigmented,congenital lesion of the skin or mucosacomposed of focal collections of roundedmelanocytes nevus cells. Nevus sebaceus syndrome nord national organization for. Duncan and coworkers1 begin their excellent paper with the unfortunate statement, linear nevus sebaceous syndrome lnss, or nevus sebaceous of jadassohn, is a rare disorder. The organoid lesion is an overgrowth of sebaceous glands in the particular area of. These nevi are classified as compound, intradermal, or junction according to the histologic pattern and location of nevus cells.
Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome. Four different tumors arising in a nevus sebaceous ncbi. The term nevus sebaceous of jadassohn was introduced in 1932, and three dermatologic stages were identified. Family practice notebook 2020, family practice notebook, llc. Basal cell carcinoma appearing in a facial nevus sebaceous of. Jadassohn was born to a jewish family living in breslau, the capital of the prussian province of silesia. Familial occurrence of nevus sebaceus of jadassohn. Department of dermatology, yale university school of medicine, usa. Most of the reported cases have occurred on the face or scalp.
A collection of disease information resources and questions answered by our genetic and rare diseases information specialists for tiechejadassohn nevus. The lesion is raised and may be pink, skin coloured or brown. This was a generation after the emancipation of the jews in central european germanspeaking lands and during a time of relative tolerance. Nevus sebaceous ns is a common congenital hamartoma of the skin. Nevus sebaceus, also called nevus sebaceus of jadassohn or organoid nevus, is a benign hamartoma of the skin, characterized by hyperplasia of the epidermis, immature hair. End of skin nonmelanocytic tumors benign nonmelanotic epidermal tumors tumorlike lesions nevus sebaceus of jadassohn this information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patients clinical data using reasonable medical. Images in clinical medicine from the new england journal of medicine nevus sebaceus of jadassohn. In 1895, jadassohn first described nevus sebaceous see the image below, a circumscribed hamartomatous lesion predominantly composed of sebaceous glands. In approximately 1020 % of the cases, different benign and malignant neoplasms develop in a preexisting nevus of jadassohn. This nevus usually appears on the face or on the scalp, and is present at birth or appears in early childhood.
Epidermal nevus en is a benign hamartomatous growth. The content is not intended to be a substitute for professional medical advice, diagnosis, or. The incidence of basal cell carcinoma and the need for prophylactic excision in children with nevus sebaceus of jadassohn have been a topic of controversy. Basal cell carcinoma appearing in a facial nevus sebaceous of jadassohn. The authors are also grateful to authors editors publishers of all. Dermoscopic analysis of nevus sebaceus of jadassohn. Trichoblastoma is the most common neoplasm developed in nevus sebaceus of jadassohn.
In the older infant, the growth of hair around the nevus accentuates the lesion. Nevus sebaceus of jadassohn is a congenital, yellowish plaque seen on the head and neck. Nevus sebaceus syndrome or schimmelpenningfeuersteinmims syndrome is a special form of nevus of jadassohn, which is usually linear and centrofacial and always affects the nose. Nevus sebaceus of jadassohn is a congenital cutaneous hamartoma comprised of multiple skin structures. First educated locally, jadassohn enrolled at the leipzig conservatory in 1848, just a few years after it had been founded by felix mendelssohn.
This rare pattern of familial occurrence prompts us to suggest that these sibs represent a case of paradominant inheritance of a mosaic disorder. Nevus sebaceus or sebaceous nevus the first term is its latin name, the second term is its name in english. Veterans administration hospital and marquette university school of dentistry blue nevus of the oral mucosa is a distinctly uncommon clinical entity. Nevus sebaceus of jadassohn is a rare cutaneous lesion over face in infancy but it is a. Nevus sebaceus, also called nevus sebaceus of jadassohn or organoid nevus, is a benign hamartoma of the skin, characterized by hyperplasia of the epidermis, immature hair follicles, and sebaceous and apocrine glands. Nord gratefully acknowledges rudolf happle, md, department of dermatology, university medical center freiburg, germany, for assistance in the preparation of this report. Syringocystadenoma papilliferum, sebaceoma, trichoblastoma, basal cell carcinoma, nevus sebaceous of jadassohn, multiple. Twelve cases in the past seven years were encountered in the.
Letters to the editor linear nevus sebaceous syndrome dear editor. Nevus sebaceus of jadassohn definition of nevus sebaceus of. Nevus sebaceous organoid nevus the dynamic natural skin care. Lansky et als1 article on the linear sebaceous nevus syndrome may be misleading to pediatricians not familiar with the dermatological nomenclature of epidermal nevi. Nevus sebaceous organoid nevus the dynamic natural. Institution of dermatology, anhui medical university, china. Nevus lipomatosus cutaneus superficialis associated with nevus sebaceous of jadassohn enver turan 1, yavuz yesilova 1, derya ucmak 2, gul turkcu 3, ozgur ilhan celik 4, mehmet salih gurel 5.
The organoid lesion is an overgrowth of sebaceous glands in the particular area of the skin and hence the names. Should nevus sebaceus of jadassohn in children be excised. Sep 05, 2018 in 1895, jadassohn first described nevus sebaceous see the image below, a circumscribed hamartomatous lesion predominantly composed of sebaceous glands. Basal cell carcinoma arising in nevus sebaceous during. In stage 1, lasting from birth to puberty, the lesion is small and hairless, and may regress in size. Basal cell carcinoma on a nevus sebaceous of jadassohn. Key points congenital skin condition which typically develops on the scalp, face, or neck caused by defective cells in the ectoderm, thought to be genetic in nature initially consists of a single. Nevus sebaceous of jadassohn or nevus sebaceous ns is a congenital hamartomatous disorder initially described by jadassohn 1 in 1895. It has the potential to develop into variety of neoplasms of various epidermal adnexal origins. Such nevi are classified as epidermal nevi and can be present at birth, or early childhood, and affect males and females of all.
Flip through the photo gallery to help identify what kind of. The tumor is hairless and often remains static until puberty when sebaceous gland. Dermis nevus sebaceous of jadassohn information on the. The sebaceous nevus is a hamartoma described by jadassohn in 1895. Skin nonmelanocytic tumor nevus sebaceus of jadassohn. If you have problems viewing pdf files, download the latest version of adobe reader for language access assistance, contact the ncats public information officer genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Nevus sebaceous or sebaceous naevus is a congenital malformation that occurs as a 12 cm plaque. Nevus sebaceous, also called nevus sebaceous of jadassohn or organoid nevus, is a benign skin malformation. While this lesion often is included in tumors of the skin adnexum, it is actually an epithelial nevus.
Nevus sebaceus of jadassohn definition of nevus sebaceus. Nevus outreach sponsors awareness programs targeted at specific specialists to improve awareness of large nevi and cmn. When checking for a misdiagnosis of nevus sebaceous of jadassohn or confirming a diagnosis of nevus sebaceous of jadassohn, it is useful to consider what other medical conditions might. The nevus sebaceus of jadassohn snj is a hamartomatous disorder of the skin and its adnexa pertaining to the group of organoid nevi, most frequently involving the face and scalp. Josef jadassohn 18631936 described this organoid condition in 1895. The role of androgen receptors in the clinical course of nevus. Nevus sebaceus of jadassohn and congenital melanocytic nevus. Defined as hyperplasia of sebaceous glands, apocrine. Linear nevus sebaceous syndrome lnss is characterized by the presence of a large, linear sebaceous nevus type of birthmark usually present from birth with a broad range. Cylindroma appearing in a preexisting nevus sebaceous. Sebaceous nevi and verrucous epidermal nevi are closely related, and many authors regard them as variants. Started in 1995, this collection now contains 6767 interlinked topic.
The features include the sebaceous nevus of jadassohn, cerebral atrophy, arachnoidal cyst, epibulbar complex choristoma, eyelid coloboma, posterior calcified scleral cartilage, and. Pharmd, phd adjunct assistant professor, university of nebraska medical center college of pharmacy. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Circumscribed lesion that occurs mainly on the face and scalp and consists predominantly of sebaceous glands, abortive hair follicles and ectopic. The authors performed a retrospective analysis of 757 cases from 1996 to 2002 in children aged 16 years or younger. Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish specific. They may be composed of a variety of epidermal cells and structures, including keratinocytes, sebaceous glands, hair follicles, apocrine and eccrine glands, and smooth muscle cells, and are thought to represent a form of cutaneous mosaicism. Nevus sebaceous of jadassohn is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Nevus sebaceous ns is a common congenital hamarto ma which. Apr 17, 2015 sebaceous naevus sn is a nonmelanocytic congenital skin hamartoma.
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