Brooke Spiegler Syndrome-A Case Report (original) (raw)

Brooke Spiegler syndrome is rare autosomal dominant hereditary disease with variable penetration. It is more common in females, usually in the second and third decades of life. The syndrome manifests with the appearance of three types of tumors: multiple cylindromas, trichoepitheliomas and spiradenomas. We presented a female patient, 54 years of age. The first changes occurred at the age of 22, first on the forehead, and then on the scalp. She has a positive family history for this condition, obtained by anamnesis (mother, two sisters, brother and daughter). We presented a dermatologic status before and after surgical intervention. Pathohistological finding of the removed tumors: cylindroma. After surgical intervention, the patient's quality of life has improved. New surgical procedures are planned.