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Pathology of Nevus Sebaceus of Jadassohn

Dr Sampurna Roy MD        

 

                                                                                                                      

 

Syn: Naevus Sebaceus  ; Organoid Naevus

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

Nevus sebaceus of Jadassohn evolve through 3 stages. (Infantile ; Puberty ; Neoplastic in adulthood). This was first described in 1965 by Mehregan and Pinkus.

If associated with features such as mental retardation, central nervous system abnormalities, oculocardiac defects, or skeletal abnormalities, it is called linear nevus sebaceous syndrome (a neurocutaneous phakomatosis).

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 hormonal changes cause proliferation and hyperplasia of the lesion. The lesion becomes verrucous with yellow lobular structures. The lesion becomes larger, verrucous and nodular.

The final stage is characterized by the appearance of nodules or tumors, with the presence of thin telangiectasias in lesions of longer evolution. Later in life, the lesions can develop benign or malignant appendageal tumors that cause further disfigurement.

Site: Located on the scalp, face or neck as a solitary lesion and 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:

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. In the late stage masses of hypertrophic sebaceous glands are present.

 

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.

 

Further  reading:

Development of six tumors in a sebaceus nevus of jadassohn: report of a case.

Basaloid tumors in nevus sebaceus revisited: the follicular stem cell marker PHLDA1 (TDAG51) indicates that most are basal cell carcinomas and not trichoblastomas.

Autosomal dominant transmission of nevus sebaceous of Jadassohn.

Apocrine carcinoma developing in a naevus sebaceous of scalp.

Nevus sebaceous at unusual location: a rare presentation.

Linear nevus sebaceous syndrome with hypophosphatemic rickets with elevated FGF-23.

Association of nevus of Jadassohn, sebaceoma and trichoblastoma in a scalp lesion.

Sebaceous nevus syndrome, central nervous system malformations, aplasia cutis congenita, limbal dermoid, and pigmented nevus syndrome.

Cylindroma appearing in a pre-existing nevus sebaceous.

Nevus sebaceous revisited.

Pedunculated lesion of the scalp with surrounding long, dark hair in a newborn. Nevus sebaceus of Jadassohn with the hair collar sign.

Proliferating trichilemmal tumor developing in nevus sebaceous.

Squamous cell carcinoma arising in a nevus sebaceous of Jadassohn in a 9-year-old girl: treatment using Mohs micrographic surgery with literature review.

Immunohistochemical study of cytokeratin expression in nevus sebaceous.

Elevated FGF-23 and parathormone in linear nevus sebaceous syndrome with resistant rickets.

Sebaceous nevus syndrome: a case report of a child with nevus sebaceus, mental retardation, seizures, and mucosal and ocular abnormalities.

Nevus sebaceus of Jadassohn revisited with reconstruction options.

Basal cell carcinoma originating from a nevus sebaceus on the scalp of a 7-year-old boy.

The role of androgen receptors in the clinical course of nevus sebaceus of Jadassohn.

Trichoblastoma is the most common neoplasm developed in nevus sebaceus of Jadassohn: a clinicopathologic study of a series of 155 cases.

 

 

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

 

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