[Basal cell nevus syndrome with sinus involvement].
The characteristics of the basal cell naevus syndrome are multiple basal cell naevi and basal cell carcinomas, cysts of the jaws, anomalies of ribs and vertebra, intracranial calcification and striking deformities of the skull, usually with a positive family anamnesis. We will discuss symptomatology, histopathology and therapy. A flemish family, where the syndrome occurs with the mother and her four children, three of whom are admitted in hospital nearly simultaneously with sinusitis maxillaris, are discussed in extenso. Also, the occurrence of the syndrome's stigmata in other members of the family is noted.
D000328:Adult; D001478:Basal Cell Nevus Syndrome / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D002280:Basal Cell Carcinoma / Q000175:diagnosis*; D005260:Female; D006801:Humans; D007573:Jaw Neoplasms / Q000175:diagnosis* / Q000235:genetics; D008297:Male; D008443:Maxillary Sinus; D008875:Middle Aged; D009808:Odontogenic Tumors / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D012852:Sinusitis / Q000209:etiology*
Het basocellulair naevussyndroom met sinusale verwikkelingen.