01793nas a2200337   4500000000100000008004100001260001300042653001000055653001100065653002100076653003800097653001100135653002500146653000900171653002300180653004600203653004400249100001600293700002000309700001400329700001800343700001200361700001800373700001600391700002200407245007000429300001100499490000700510520092400517022001401441       2013                            d  c2013 Feb10aAdult10aBrazil10aGenes, Recessive10aGenetic Predisposition to Disease10aHumans10aLeprosy, lepromatous10aMale10aNetherton Syndrome10aProteinase Inhibitory Proteins, Secretory10aSerine Peptidase Inhibitor Kazal-Type 51 aOliveira FL1 aVasconcellos BO1 aMorais TS1 aNascimento MB1 aTeles R1 aCosta Nery JA1 aMiranda MJS1 aAzulay-Abulafia L00aNetherton's syndrome and lepromatous leprosy: a mere coincidence?  a186-900 v523 a<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>  a1365-4632