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Syn: Naevus Sebaceus of Jadassohn ; Organoid Naevus

Nevus sebaceus of Jadassohn is a benign, congenital hamartoma of the folliculo-sebaceous apocrine unit and epidermis that often presents at birth, appears to regress in childhood, and grows during puberty, suggesting possible hormonal control.

Clinical presentation:

In childhood, the lesion consists of a circumscribed hairless yellow-orange-colored, waxy, pebble-like, papule or plaque  often linear or round or irregular. In puberty the lesion becomes verrucous and nodular.

Site: Located on the scalp, face or neck as a solitary lesion & usually present since birth.

Nevus sebaceous may develop tumours in adulthood particularly, syringocystadenoma papilliferum and benign hair follicle tumours. Basal cell carcinoma has been observed in about 5 percent cases.

Microscopic features:

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Histologically, there is verrucous epidermal hyperplasia together with immature sebaceous gland and basaloid hyperplasia. Immature hair structures and dilated infundibulum are present. Ectopic apocrine glands are also noted.
The sebaceous glands are well developed in the first few months of life, through childhood sebaceous glands are underdeveloped and in adolescence these are reduced in number and size.

Differential diagnosis:   Sebaceous hyperplasia ;  Seborrheic keratosis.
It may be difficult to differentiate between basal cell carcinoma and basaloid proliferation that arise in malformed hair germs in nevus sebaceous.

Nevus sebaceus of Jadassohn revisited with reconstruction options.Int J Dermatol. 2005;44(2):145-50.

Follicular hybrid cyst (trichilemmal cyst and pilomatricoma) arising within a nevus sebaceus.Am J Dermatopathol. 2004 ;26(5):390-3.

Sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum arising within a nevus sebaceus.Dermatol Surg. 2004 ;30(12 Pt 2):1546-9.

Should nevus sebaceus of Jadassohn in children be excised? A study of 757 cases, and literature review. J Craniofac Surg. 2003 ;14(5):658-60.

Incidence of cytokeratin 20 expressed cells in primitive follicular structure and secondary neoplastic proliferations of nevus sebaceus. J Dermatol Sci. 2003 ;33(1):17-21.

Apocrine carcinoma developed in nevus sebaceus of Jadassohn. Eur J Dermatol. 2003 ;13(5):487-9.

Mucoepidermoid carcinoma arising within nevus sebaceus of Jadassohn. J Cutan Pathol 2003;30:652-655.

Sebaceus naevi: a clinicopathologic study. J Eur Acad Dermatol Venereol. 2002 ;16(4):319-24.

Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.Pathol Res Pract 2001;197(4):279-84.

Desmoplastic neuroepithelial tumor of infancy in the nevus sebaceus syndrome: report of a unique constellation and review of the literature.Pathol Res Pract 2001;197(4):279-84.

Basal cell carcinoma originating from a nevus sebaceus on the scalp of a 7-year-old boy.J Pediatr Hematol Oncol 2001 ;23(4):247-9.

The role of androgen receptors in the clinical course of nevus sebaceus of Jadassohn.Mod Pathol 2001;14(6):539-42.

Segmental neurofibromatosis in association with nevus sebaceus of Jadassohn. J Am Acad Dermatol 2000;43(5 Pt 2):895-7.

Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases. Am J Dermatopathol 2000;22(2):108-18.

Tumors arising in nevus sebaceus: A study of 596 cases.J Am Acad Dermatol 2000;42(2 Pt 1):263-8.

Sebaceus nevus of Jadassohn. Apropos of 62 surgically treated cases and review of the literature. Rev Stomatol Chir Maxillofac. 1999 ;100(4):175-9.

Spiradenoma arising in a nevus sebaceus of Jadassohn: case report and literature review.Am J Dermatopathol 1999 ;21(5):462-7.

Tubular apocrine carcinoma arising in a nevus sebaceus of Jadassohn. Am J Dermatopathol 1998;20(4):389-92.

A new neurocutaneous syndrome: nevus sebaceus syndrome.Cutis 1993;51(6):437-41.

Basaloid neoplasms in nevus sebaceus. J Cutan Pathol. 2000 ;27(7):327-37.

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