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To the Editor: Gorlin syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare multisystemic genodermatosis classically characterized by the development of numerous basal cell carcinomas (BCC)1,2,3. Gorlin syndrome is caused by germline inactivating mutations in the patched ( PTCH ) gene, resulting in constitutively active hedgehog signaling1,2. The hedgehog signaling pathway is important in the regulation of stem cell and progenitor cell proliferation, the maintenance and regeneration of adult tissues, and finally in carcinogenesis, including the development of sporadic BCC2,4. Vismodegib, an oral inhibitor of hedgehog signaling, has demonstrated efficacy in Gorlin syndrome, as well as in patients with locally advanced or metastatic BCC2,4.Recently, dysregulated hedgehog signaling has also been implicated in the pathogenesis of rheumatoid arthritis (RA). Hedgehog signaling is highly active in the synovium of patients with RA in vivo , as well as in cultured fibroblast-like synoviocytes (FLS) from patients in vitro 5,6. In a mechanistically similar fashion to the malignant keratinocytes within … Address correspondence to Dr. J.F. Scott, University Hospitals Case Medical Center, 11100 Euclid Ave., Cleveland, Ohio 44106, USA. E-mail: jeffrey.scott{at}uhhospitals.org

Severe Rheumatoid Arthritis Developing in Conjunction with Gorlin Syndrome