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Indications and limitations of afamelanotide for treating vitiligo-reply.

fibrofolliculoma, and the histopathologic distinction is related to differences in sectioning techniques.2 Fibrofolliculomas are characterized by anastomosing epithelial strands radiating from a dilated hair follicle within a fibrous stroma. Birt-Hogg-Dubé syndrome has an autosomal dominant pattern of inheritance and is caused by germline mutations in the FLCN gene, which is located on chromosome 17p11.2. This gene encodes for the folliculin protein, the role of which has not been fully elucidated; however, it appears to function as a tumor suppressor. Studies have found that folliculin interacts with FLCNinteracting protein 1 and is involved in 5′-AMP activated protein kinase and mTOR signaling dysregulation.3 Renal tumors and spontaneous pneumothoraces are strongly linked to BHDS. There is a 50-fold increased risk of spontaneous pneumothorax, and recurrent disease is common. Pneumothorax is probably related to the presence of multiple lung cysts, which have been reported in more than 80% of adult patients and are typically found at the lung bases.4 Patients with BHDS have a 16% risk of developing renal cell carcinoma by age 70 years, which is often bilateral and multifocal.5 Chromophobe renal carcinoma and oncocytomas are most commonly reported. Although there is no consensus on screening guidelines, renal magnetic resonance imaging surveillance beginning at age 20 years has been recommended.1 Treatment for the cutaneous manifestations of BHDS is difficult. Multiple modalities, including ablative lasers, electrodessication, and surgical excision have been reported. While surgical excision is the only definitive treatment for individual lesions, the scarring risk makes this an unreasonable option for multiple lesions. A recent double-blind placebocontrolled randomized trial using topical rapamycin yielded disappointing results.6 Most importantly, appropriate imaging studies for renal tumors and lung cysts should be obtained in all patients with BHDS as well as at-risk relatives. To our knowledge, this patient is the first reported case of BHDS in an individual of African ancestry and is associated with an FLCN gene mutation that has not been previously described.