Chronic mucocutaneous or invasive fungal infections are generally the result of primary or secondary immune dysfunction. Patients with autosomal recessive CARD9 mutations are also predisposed to recurrent mucocutaneous and invasive fungal infections with Candida spp., dermatophytes (e.g., Trichophyton spp.) and phaeohyphomycetes (Exophiala spp., Phialophora verrucosa). We study a consanguineous family of Turkish origin in which three members present with distinct clinical phenotypes of chronic mucocutaneous and invasive fungal infections, ranging from chronic mucocutaneous candidiasis (CMC) in one patient, treatment-resistant cutaneous dermatophytosis and deep dermatophytosis in a second patient, to CMC with Candida encephalitis and endocrinopathy in a third patient. Two patients consented to genetic testing and were found to have a previously reported homozygous R70W CARD9 mutation. Circulating IL-17 and IL-22 producing T cells were decreased as was IL-6 and granulocyte/macrophage colony-stimulating factor (GM-CSF) secretion upon stimulation with Candida albicans. Patients with recurrent fungal infections in the absence of known immunodeficiencies should be analyzed for CARD9 gene mutations as the cause of fungal infection predisposition.
Background: Limited data are available about the use of 308-nm monochromatic excimer light (MEL) and localized 311-nm narrow-band ultraviolet B (NB-UVB) in the treatment of vitiligo. The aim of this study was to evaluate the efficacy of 308-nm MEL versus localized 311-nm NB-UVB in vitiligo patients. Methods: Eleven patients participated in this prospective intrapatient placebo-controlled randomized trial. In each patient, 3 lesions were selected and treated with NB-UVB, MEL and placebo during 24 sessions, respectively. Repigmentation was evaluated clinically and by objective surface measurement. Results: Twenty percent of the lesions treated with NB-UVB achieved repigmentation scores above 50%. None of the lesions treated with MEL achieved a repigmentation higher than 50% after 24 sessions. Conclusion: Localized 311-nm NB-UVB is effective in the treatment of vitiligo. It should be considered for localized vitiligo as it is easily accessible. In this study the efficacy of localized 311-nm NB-UVB was superior to 308-nm MEL.
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