Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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Stuart R. Lessin and Clifford S. Perlis
Evidence Levels: A Double-blind study B Clinical trial ≥ 20 subjects C Clinical trial < 20 subjects D Series ≥ 5 subjects E Anecdotal case reports
Nevus sebaceus, first described by Jadassohn in 1895, is a term for a congenital hamartoma of the epidermis and adnexal structures typically involving the scalp and face. It presents at birth or appears in early childhood as a pink, orange to yellow waxy plaque with a granular pitted surface that is often hairless. The size and configuration can be variable. During puberty, the lesion thickens and becomes verrucous as glands enlarge within the dermis. Development of cutaneous and adenexal neoplasms has been reported in 10–20% of lesions after puberty and in adulthood. These neoplasms are most commonly benign. Surgical treatment addresses cosmetic issues, as well as prophylaxis or treatment of neoplasms.
For most lesions, clinical examination is sufficient to establish the diagnosis. A skin biopsy can confirm the clinical impression when indicated. Most neoplastic growths that arise in a nevus sebaceous do not develop until after the age of 16 years; the overwhelming majority of cases are benign. These include syringocystadenoma papilliferum, trichoblastoma, tricholemmoma, sebaceoma, nevocellular nevus, and seborrheic keratosis. The most common malignancy that develops within a nevus sebaceous is a basal cell carcinoma, but the absolute incidence is very rare. Isolated case reports describe rare patients who have developed sebaceous carcinoma, squamous cell carcinoma, tricholemmal carcinoma or microcystic adnexal carcinoma within nevus sebaceus.
In 2000, a case series established that trichoblastoma was the most common neoplasm arising in nevus sebaceus. With this evidence of minimal risk of malignant neoplastic transformation, prophylactic surgery is not justified. Conservative management with clinical observation and biopsy of any lesions suspicious for malignancy appears to be most prudent.
Cribier B, Scrivener Y, Grosshans E. J Am Acad Dermatol 2000; 42(2 Pt 1): 263–8.
This is a retrospective case series of 596 cases demonstrating a 1.7% occurrence of benign tumors in childhood. Most tumors arising in nevus sebaceus occurred in adults over 40 years; in these adults, 2.1% of the neoplasms were basal cell carcinomas. The authors conclude that prophylactic surgery in children is of uncertain benefit.
Santibanez-Gallerani A, Marshall D, Duarte AM, Melnick SJ, Thaller S. J Craniofac Surg 2003; 14: 658–60.
This is a retrospective case series of 757 cases of children aged 16 years or younger. No cases of basal cell cancer were found. The authors questioned the need for prophylactic excision in children.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
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