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Published on: July 15, 2011
Netherton's syndrome and lepromatous leprosy: a mere coincidence?
Felipe Ladeira de Oliveira1, Bruna Obeica Vasconcellos, Thais Schiavo de Morais
1Section for Sexually Transmitted Diseases and Leprosy, Professor RD Azulay Institute of Dermatology, Hospital Santa Casa da Misericórdia, Rio de Janeiro, Brazil.
Objectives:
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.
Methods:
This paper represents the first case report of leprosy and concurrent NS.
Discussion:
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.
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