LAUSR

Dear Editor, Vismodegib, a first-in-class inhibitor of the hedgehog signaling pathway, is indicated to treat metastatic or locally advanced basal cell carcinoma (BCC). Mutations in Patched 1 (PTCH1) and smoothened (SMO) proteins of the Hedgehog (Hh) pathway appear to be the most common occurrences in BCC. Vismodegib acts as a small molecule inhibitor of SMO, a transcription gene present in the Hedgehog signaling molecule pathway and implicated in the proliferation of BCCs. The Hh pathway is abnormally activated in patients with both sporadic and inherited BCCs (Gorlin syndrome or BCC nevus syndrome), and its inhibition results in significant clinical responses (tumor shrinkage or complete remission). Gorlin syndrome, is a rare, autosomal-dominant, multisystem disorder with an estimated prevalence of around 1 in 100 000 on average. This syndrome is diagnosed clinically with major criteria, including multiple BCCs, odontogenic keratocysts of the jaw, palmar/plantar pits, falx cerebri calcifications, rib deformities, presence of a diagnosis of Gorlin-Goltz syndrome in a first-degree relative and minor criteria, like medulloblastomas, ovarian fibroma, macrocephaly, skeletal anomalies, and congenital malformations. A multidisciplinary approach is necessary in the diagnosis and treatment of these patients. One of the challenges regarding the use of vismodegib is its classrelated pharmacological toxicity, including amenorrhea. Recently, the Hh pathway has been shown to play an important role in ovarian steroidogenesis. We report the case of a 40-year-old nulliparous woman with Gorlin-Goltz syndrome who had previously undergone multiple surgeries. On clinical examination, she presented several BCCs in the head-neck region and on the upper and lower limbs. She had an excellent response to vismodegib: as early as the second month of therapy her BCC skin lesions decreased considerably in size. Furthermore, she did not develop any new skin lesions. Three months from the beginning of vismodegib therapy, she became amenorrheic. The pregnancy test was negative. No pain-related bleeding or secretion losses were