Introduction
Tuberous sclerosis complex (TSC), an autosomal dominant genetic disorder
caused by TSC1 or TSC2 mutations is characterized by hamartomas in
various organs (e.g., skin, brain, lungs, and kidneys) and appears with
an incidence rate of approximately 1 in 5000–10,000.1Angiofibromas are the most frequent skin lesions occurred in patients
with TSC older than 5 years and characteristically consist of numerous
pink to reddish papules or nodules that are typically located on the
cheeks, nose, and chin.2 The traditional and current
treatment modalities for facial angiofibromas include topical sirolimus,
topical rapamycin cryosurgery, curettage, dermabrasion, chemical
peeling, excision, and laser therapies. Treatment options generally vary
according to the clinical presentation of the disease and the location
of the TSC related lesions. Treatments for skin lesions in the face and
head and neck area are usually intended for cosmetical issues, except
for ocular manifestations, and the treatment decision is made according
to the severity of the disease (e.g. Facial Angiofibroma Severity
Index). Auricula and Meatus Acusticus Externus (MAE) involvement in TSC
related angiofibroma a rare and specific pathology due to the aesthetic
and functional feature of the ear.