Among the 3 variants of blue nevi, the cellular variant occurs less frequently than the. Nevus sebaceous of jadassohn is a common skin lesion of children. Jadassohn, josef, german dermatologist in switzerland, 18631936. 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. Sebaceous nevi and verrucous epidermal nevi are closely related, and many authors regard them as variants. Nevus sebaceous is also known as an organoid nevus and nevus sebaceus of jadassohn. Dermweb is operated by the department of dermatology and skin science at the university of british columbia, vancouver, bc, canada. Posterior scleral choristoma in the organoid nevus syndrome linear nevus sebaceus of jadassohn. A lesion of nevus sebaceus with multifocal basalcell carcinomas was excised by mohs freshtissue tech.
Nevus comedonicus an overview sciencedirect topics. Jadassohn nevus phakomatosis symptoms, causes, diagnosis, and treatment information for jadassohn nevus phakomatosis nevus sebaceous of jadassohn with alternative diagnoses, fulltext book chapters, misdiagnosis, research treatments, prevention, and prognosis. Jadassohntieche nevus definition of jadassohntieche. Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome. Nevus sebaceous of jadassohn ns is a hamartoma that affects the epidermis, ectopic eccrine and apocrine glands and hair follicles. Basal cell carcinoma appearing in a facial nevus sebaceous.
Basal cell carcinoma appearing in a facial nevus sebaceous of jadassohn. Josef jadassohn 18631936 described this organoid condition in. Encyclopedia article about jadassohn nevus by the free dictionary. Nevus sebaceus of jadassohn definition of nevus sebaceus. Reviews the deep penetrating nevus lauren strazzula, ba,a maryanne makredes senna, md,b mariko yasuda, md,b and leah belazarian, mdb,c worcester, massachusetts the deep penetrating nevus dpn, also known as the plexiform spindle cell nevus, is a pigmented lesion that commonly arises on the head and neck in the first few decades of life. At birth or in early infancy, nevus sebaceus appears as a hairless, solitary, linear or round, slightly raised, pinkish, yellow, orange, or tan plaque, with a smooth or somewhat velvety surface. Pdf nevus sebaceous also known as nevus sebaceous of jadassohn ns is an epidermal nevi predominantly congenital sebaceous. In approximately 1020 % of the cases, different benign and malignant neoplasms develop in a preexisting nevus of jadassohn. Nevus sebaceous of jadassohn jama pediatrics jama network. Nevus sebaceous of jadassohn is listed as a rare disease by the office of rare diseases ord of the national institutes of health nih. Patients should address specific medical concerns with their physicians. Linear nevus sebaceous syndrome is reported to occur in as many as 1 in live births, and is hypothesized to result from genetic mosaicism involving a lethal autosomaldominant gene.
Most of the nevi are situated on the scalp and correspond to the type called nevus sebaceus of jadassohn. Family practice notebook 2020, family practice notebook, llc. No cases of basal cell cancer were found in the nevus sebaceus group. Nevus sebaceous is a congenital skin condition which typically appears on the scalp, but may also develop on the face or neck.
Nevus sebaceus passes through 3 clinically distinct stages, as follows. The blue nevus is found most frequently on the skin. If you have problems viewing pdf files, download the latest version of adobe reader. Nevus sebaceous organoid nevus the dynamic natural. Onethird of scap cases are associated with nevus of jadassohn. Nearly all lesions occur on the scalp, forehead or face and appear clinically as smooth yellowish, hairless patches. Present at birth, the condition initially consists of a single. Nevus sebaceous, also called nevus sebaceous of jadassohn or organoid nevus, is a benign skin malformation. Monarchs tools are designed to make it easier to compare the signs and symptoms phenotypes of different diseases and.
Nevus lipomatosus cutaneus superficialis associated with. Individuals with ns may develop benign or malignant tumors, with increasing evidence. Lesions are usually present at birth and appear as waxy, yelloworange or tan, hairless plaques. Manual of histologic and special staining technics, ed 2, new york. Basal cell carcinoma on a nevus sebaceous of jadassohn. Borstjadassohn type intraepidermal epithelioma see under borst franceschettijadassohn syndrome synonyms. Nevus sebaceous is a hallmark of schimmelpenning syndrome, and if. Development of six tumors in a sebaceus nevus of jadassohn. Nevus sebaceus of jadassohn how is nevus sebaceus of. Neuroradiological findings in jadassohn nevus phakomatosis. Nevus sebaceus or sebaceous nevus the first term is its latin name, the second term is its name in english. Definition of nevus, sebaceous of jadassohn in the dictionary. Multifocal basalcell carcinomas in a nevus sebaceus of jadassohn morris westfried, m.
Key points congenital skin condition which typically develops on the scalp, face, or neck. Rosario serrano a, jose luis rodriguezperalto a, daniel azorin a. Dermis nevus sebaceous of jadassohn information on the. The organoid lesion is an overgrowth of sebaceous glands in the particular area of the skin and hence the names.
Naegeli syndrome jadassohn nervus congenital papillary acanthosis of the epidermis, with hyperplasia of sebaceous glands developing at puberty and presence of apocrine glands. Nevus sebaceus of jadassohn is a congenital abnormality first described by the dermatologist josef jadassohn in 1895. A paracrinopathy with variable phenotype, american journal of medical genetics part a on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. Should nevus sebaceus of jadassohn in children be excised. Basal cell carcinoma arising in nevus sebaceous during.
Organoid nevus nevus sebaceus of jadassohn is a complex hamartoma involving not only the pilosebaceous follicle, but also the epidermis, and often other adnexal structures. The terms epidermal nevus syndrome and linear nevus sebaceous syndrome are often used interchangeably, although linear nevus sebaceous syndrome more strictly applies to patients. The common blue nevus is the second most common variant. Nevus sebaceous of jadassohn is a congenital sebaceous gland hamartoma usually found on the scalp and face. Intramucosal nevi make up more than one half of all reported intraoral melanocytic nevi. Jadassohn nevus article about jadassohn nevus by the. Nevus sebaceus of jadassohn nsj is a benign, congenital hamartoma that often presents at birth, appears to regress in childhood, and. Nevus sebaceous or sebaceous naevus is a congenital malformation that occurs as a 12 cm plaque. Skin nonmelanocytic tumor nevus sebaceus of jadassohn. The nevus usually is located on the face, scalp, or neck. Circumscribed lesion that occurs mainly on the face and scalp and consists predominantly of sebaceous glands, abortive hair follicles and ectopic apocrine glands. Nevus or nevi if multiple is a nonspecific medical term for a visible, circumscribed, chronic lesion of the skin or mucosa. Oculocerebral dysgenesis in the linear nevus sebaceous.
Basal cell carcinoma appearing in a facial nevus sebaceous of. Present at birth, the condition initially consists of a single hairless patch of orangeyellow skin. The epidermal changes are variable but usually there is acanthosis and sometimes mild papillomatous hyperplasia fig. During adolescence, the lesion may become warty, bumpy or scaly. In the older infant, the growth of hair around the nevus accentuates the lesion. 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 how is nevus sebaceus of jadassohn abbreviated. Nevus sebaceous of jadassohn or nevus sebaceous ns is a congenital hamartomatous disorder initially described by jadassohn 1 in 1895. It starts as a flat pink or orange plaque slightly raised area. Multifocal basalcell carcinomas in a nevus sebaceus of. Dermoscopic analysis of nevus sebaceus of jadassohn. Four patients with jadassohn nevus phakomatosis linear nevus sebaceus syndrome, schimmelpenningfeuerstein. Pdf nevus sebaceous of jadassohn a rare case report. Nevus sebaceus of jadassohn ns or organoid nevus is a complex cutaneous hamartoma involving not only the pilosebaceus follicle but also the epidermis and often other adnexal structures.
The role of androgen receptors in the clinical course of nevus. This means that nevus sebaceous of jadassohn, or a subtype of nevus sebaceous of jadassohn, affects less than 200,000 people in. The monarch initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Pdf on aug 3, 2015, kelly segars and others published nevus sebaceus of jadassohn find, read and cite all the research. Cylindroma appearing in a preexisting nevus sebaceous. Presented in part as a poster at the american academy of. There are several areas of interest for the general public. The authors performed a retrospective analysis of 757 cases from 1996 to 2002 in children aged 16 years or younger. It may also appear on the face but this is less common. A nevus sebaceous is a type of birthmark that usually appears on the scalp. In 1895, jadassohn first described nevus sebaceous see the image below, a circumscribed hamartomatous lesion predominantly composed of sebaceous glands. Even though present at birth, it is more readily seen at 1 month to 6 weeks of age.
Association of nevus of jadassohn, sebaceoma and trichoblastoma in a scalp lesion. To describe different dermoscopic patterns of this nevus. Information and translations of nevus, sebaceous of jadassohn in the most comprehensive dictionary definitions resource on the web. Its transition to squamous cell carcinoma is a rare entity. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. There is variable hyperpigmentation of the basal layer with some. Nevus sebaceus of jadassohn nsj is a complex cutaneous. Common terms, including mole, birthmark, and beauty mark, are used to describe nevi, but these terms do not distinguish. The changes may resemble those seen in an epidermal nevus see p. Posterior scleral choristoma in the organoid nevus syndrome linear. 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. We describe two sibs a boy and a girl suffering from linear nevus sebaceus of jadassohn. Twelve cases in the past seven years were encountered in the.
Although access to this page is not restricted, the information found here is intended for use by medical providers. This rare pattern of familial occurrence prompts us to suggest that these sibs represent a case of paradominant inheritance of a mosaic disorder. The topic nevus sebaceous of jadassohn you are seeking is a synonym, or alternative name, or is closely related to the medical condition nevus sebaceous. Estas lesiones pueden alcanzar gran tamano y provocan en ocasiones una importante deformidad estetica. Familial occurrence of nevus sebaceus of jadassohn. Such nevi are present at birth, or early childhood, affecting males and females of all races equally. Linear nevus sebaceous syndrome lnss is a condition characterized by the association of a large, linear sebaceous nevus type of birthmark with a broad range of abnormalities that may affect every organ system, including the central nervous system cns.543 1405 712 1095 868 368 414 693 381 1332 479 874 292 98 714 744 883 232 1493 1078 892 30 306 60 923 24 157 588 140 230 1079 330 645 106 174 861 1266 261 169 976 41 1112 961 891 729 408 159 1078