Nevus sebaceous syndrome

Autor: Kee Woan Park, Si‐Yong Kim, Jung Min Choi, In Gang Jang
Rok vydání: 1999
Předmět:
Zdroj: International Journal of Dermatology. 38:531-533
ISSN: 1365-4632
0011-9059
DOI: 10.1046/j.1365-4362.1999.00693.x
Popis: A 10-day-old boy presented with an 11-cm × 7-cm asymptomatic well-demarcated alopecic verrucous yellowish plaque on the right temporoparietal area, a 5-cm × 3-cm verrucous yellowish plaque on the forehead, and a linear plaque extending from the forehead to the nose ridge present since birth. He also had a 2-cm pedunculated mass from the right bulbar and tarsal conjunctiva ( Fig. 1). The gestational age was 39 weeks and 5 days and he weighed 4.04 kg at birth. No abnormalities were observed upon physical examination except for the cutaneous and optical findings. An examination of the fundus revealed macular agenesis and peripapillary atrophy ( Fig. 2). No family history was revealed. The results of laboratory tests, including complete blood count, blood chemistry, immunochemical study, screening test for inborn metabolic disorders (methionine, leucine, phenylalanine, galactose, thyroid hormone, thyroid stimulating hormone), and antibody tests to TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes virus), were normal. The results from the chromosomal study, brainstem auditory evoked potential, and visual evoked potential were normal. Chest and skull X-ray did not show any abnormal signs. Orbit and brain magnetic resonance images showed findings suggesting a lipodermoid cyst from the right eyeball and subdural hygroma or arachnoid cysts in both of the frontal and both of the temporal areas ( Fig. 3). The histopathologic examination of a lesional biopsy specimen revealed hyperkeratosis, papillomatosis in the epidermis, large numbers of sebaceous glands of various sizes, and immature hair structures in the dermis. Figure 1. Asymptomatic verrucous yellowish alopecic plaque on the right frontal temporoparietal area and linear verrucous yellowish plaque on the nose. Solitary 2-cm pedunculated mass from right tarsal and bulbar conjunctiva Download figure to PowerPoint Figure 2. Fundus photograph reveals macular agenesis and peripapillary atrophy Download figure to PowerPoint Figure 3. Widening of the cerebrospinal fluid (CSF) space is noted at the bilateral anterior temporal fossa and bilateral frontal area, suggesting subdural hygroma or arachnoid cyst Download figure to PowerPoint
Databáze: OpenAIRE
Externí odkaz: