Brooke-Spiegler Syndrome (BSS) is a rare disease manifesting as development of multiple trichoepitheliomas secondary to mutation of the CYLD gene. As a result, the skin acquires irregular deformities that is cosmetically unpleasant. This case report showcases a patient with gene mapping confirmed diagnosis of BSS, with the confluent lesions causing nasal airway obstruction. The patient was treated with surgical excision, followed by reconstruction using paramedian forehead flap. The paramedian forehead flap has long been indicated for reconstructing nasal defects primarily secondary to malignancies. As of writing, the patient presented with a viable flap, satisfactory healing of the donor site, no evidence of disease recurrence in the post operative site, and to our observation, a decrease in the number of lesions transferred from the flap to the defect. The primary indication for intervention is the disfiguring nature of the disease. While the current pool of knowledge offers diverse options for treatment, surgical excision remains as the gold standard. With the genetic mutation basis of the disease, the authors recommend close follow-up of the patient for possible recurrence.

Ariele D Laudencia is a graduate doctor of medicine at Pamantasan ng Lungsod ng Maynila, Philippines. She is a medical officer III, and resident in-training of the department of otorhinolaryngology - head and neck surgery at Ospital ng Maynila Medical Center.