TY - JOUR
KW - Adult
KW - Brazil
KW - Genes, Recessive
KW - Genetic Predisposition to Disease
KW - Humans
KW - Leprosy, lepromatous
KW - Male
KW - Netherton Syndrome
KW - Proteinase Inhibitory Proteins, Secretory
KW - Serine Peptidase Inhibitor Kazal-Type 5
AU - Oliveira FL
AU - Vasconcellos BO
AU - Morais TS
AU - Nascimento MB
AU - Teles R
AU - Costa Nery JA
AU - Miranda MJS
AU - Azulay-Abulafia L
AB - <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>
BT - International journal of dermatology
C1 - http://www.ncbi.nlm.nih.gov/pubmed/23347305?dopt=Abstract
DA - 2013 Feb
DO - 10.1111/j.1365-4632.2012.05620.x
IS - 2
J2 - Int. J. Dermatol.
LA - eng
N2 - <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>
PY - 2013
SP - 186
EP - 90
T2 - International journal of dermatology
TI - Netherton's syndrome and lepromatous leprosy: a mere coincidence?
VL - 52
SN - 1365-4632
ER -