Sitagliptin does not improve fibrosis score or NAS after 24 wk of therapy. The MRI IDEAL technique may be useful for non-invasive measurement of hepatic steatosis.
Although the exact pathogenesis of vitiligo is uncertain, each of these theories likely plays a role. Understanding each theory would pave the way for therapeutic advances for this disease.
Relapsing polychondritis, or RP, is a rare connective tissue disease characterized by relapsing-remitting destructive inflammation of the cartilaginous and other proteoglycan-rich structures in the body. Given the relatively low incidence of RP, a concise clinically relevant guide, focusing on the cutaneous manifestations of this serious disease, is lacking. In this review, we provide the dermatologist with an approach to diagnosing RP and a guide to its initial work-up, and management. We close with an overview of the currently available treatment modalities for RP.
Neurofibromatosis type I (NF1) is an autosomal dominant condition caused by mutations in the tumor suppressor NF1-encoded protein, neurofibromin, which normally negatively regulates the mammalian target of rapamycin (mTOR) pathway. Such mutations clinically lead to the development of a variety of tumors including cutaneous neurofibromas (NFs), plexiform neurofibromas, and malignant peripheral nerve sheath tumors. 1 Sirolimus, which inhibits the mTOR pathway, has been shown to be effective in managing tumor size and associated symptoms of plexiform neurofibromas (PNs) in patients with NF1. 2-4 Abstract A 14-year-old boy with neurofibromatosis type I (NF1) presented with a painful neurofibroma on his right palm. The lesion was treated with topical sirolimus, resulting in decreased size and pain and improvement in motor function of his hand. This case demonstrates the efficacy of topical sirolimus in the management of neurofibromas in NF1. K E Y W O R D S neurofibroma, neurofibromatosis type 1, sirolimus | 361 Pediatric Dermatology MALHOTRA eT AL.
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