@article{23882,
  keywords = {Adult, Brazil, Genes, Recessive, Genetic Predisposition to Disease, Humans, Leprosy, lepromatous, Male, Netherton Syndrome, Proteinase Inhibitory Proteins, Secretory, Serine Peptidase Inhibitor Kazal-Type 5},
  author = {Oliveira FL and Vasconcellos BO and Morais TS and Nascimento MB and Teles R and Costa Nery JA and Miranda MJS and Azulay-Abulafia L},
  title = {Netherton's syndrome and lepromatous leprosy: a mere coincidence?},
  abstract = {<p><b>OBJECTIVES: </b>Netherton's syndrome (NS) is a rare autosomal recessive condition, first described in 1958, which involves a complex immunological dysfunction, ichthyosiform dermatitis, and erythroderma, characteristic defects of the hair shaft and atopy. Recurrent bacterial infection in the skin of patients with NS is frequent.</p><p><b>METHODS: </b>This paper represents the first case report of leprosy and concurrent NS.</p><p><b>DISCUSSION: </b>This case merits discussion among doctors in endemic and non-endemic areas to evaluate the chronic use of systemic corticosteroids as a risk factor for leprosy. The present patient came from an endemic area of leprosy and was treated chronically with systemic corticosteroids for erythroderma. This treatment, along with the immunodeficiency related to the syndrome and caused by a genetic mutation in SPINK5, may be a facilitating factor for the infection.</p>},
  year = {2013},
  journal = {International journal of dermatology},
  volume = {52},
  pages = {186-90},
  month = {2013 Feb},
  issn = {1365-4632},
  doi = {10.1111/j.1365-4632.2012.05620.x},
  language = {eng},
}