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Publication

Netherton's syndrome and lepromatous leprosy: a mere coincidence?

Abstract

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.

More information

Type
Journal Article
Author
Oliveira FL
Vasconcellos BO
Morais TS
Nascimento MB
Teles R
Costa Nery JA
Miranda MJS
Azulay-Abulafia L
Year of Publication
2013
Journal
International journal of dermatology
Volume
52
Issue
2
Number of Pages
186-90
Date Published
2013 Feb
Language
eng
ISSN Number
1365-4632
DOI
10.1111/j.1365-4632.2012.05620.x
Alternate Journal
Int. J. Dermatol.
Publication Language
eng