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Schimmelpenning–Feuerstein–Mims Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Cases related to SFM syndrome (also known as Schimmelpenning syndrome, nevus sebaceus syndrome, linear sebaceous nevus sequence, nevus sebaceous of Jadassohn, Jadassohn nevus phacomatosis, Jadassohn sebaceous nevus syndrome, organoid nevus, and epidermal nevus syndrome) were initially described by Jadassohn in 1895. Further studies by Schimmelpenning in 1957 and Feuerstein and Mims in 1962 defined this disorder by the classic triad of symptoms (nevus sebaceous, epilepsy, and mental retardation). Since then, additional anomalies (including neurologic, ophthalmic, skeletal, cardiovascular, and urologic defects) have been reported from patients with SFM syndrome, which makes diagnosis of this disorder on the basis of classic triad inadequate [2].
Principles of Clinical Diagnosis
Published in Susan Bayliss Mallory, Alanna Bree, Peggy Chern, Illustrated Manual of Pediatric Dermatology, 2005
Susan Bayliss Mallory, Alanna Bree, Peggy Chern
Size ranges from 0.5 to >6 cm; can be extensive In childhood, the lesions flatten and become less apparent; thought to be caused by the reduction of maternal hormones and sebaceous glandsAt puberty, most plaques thicken and become verrucous (Figure 17.5)Development of hamartomatous tumors within the lesion usually after pubertyTumors: syringocystadenoma papilliferum, trichoblastoma, hidradenoma, basal cell carcinoma, othersMalignant tumors such as squamous cell carcinoma, apocrine carcinoma and other adnexal carcinomas are rare before age 40 yearsDetected by development of a papule with ulceration within the lesionExtensive lesions may be associated with multiple systemic manifestations in the nevus sebaceus syndrome (Schimmelpenning syndrome): seizures, mental retardation, arteriovenous malformations of the brain, skeletal abnormalities, ophthalmologic abnormalities
Basal cell carcinoma within nevus sebaceous of the trunk
Published in Baylor University Medical Center Proceedings, 2019
Ian T. Watson, Andrew DeCrescenzo, So Yeon Paek
An 82-year-old woman presented to the dermatology outpatient clinic for full-body skin examination. An irregularly shaped, approximately 18 cm × 8 cm, light brown verrucous plaque in a Blaschkoid pattern was identified on the right upper back (Figure 1). The patient stated that the lesion had been present since birth and denied symptoms. Upon dermoscopic evaluation, several pearly papules were appreciated within the large plaque. Shave biopsy of a suspicious papule within the plaque revealed nodular basal cell carcinoma (BCC) on histopathology (Figure 2a). Background NS was also noted on histopathology (Figure 2b). Additional biopsies taken within the lesion further demonstrated nodular and superficial BCCs, trichilemmoma, and tumor of the follicular infundibulum with background NS. In total, five BCCs were diagnosed within this patient’s NS, which were treated with elliptical excision and topical imiquimod. She did not demonstrate additional features of Schimmelpenning syndrome or phakomatosis pigmentokeratotica.