Conflicts of interest: None.
Tropical medicine rounds
Netherton’s syndrome and lepromatous leprosy: a mere coincidence?
Article first published online: 24 JAN 2013
© 2013 The International Society of Dermatology
International Journal of Dermatology
Volume 52, Issue 2, pages 186–190, February 2013
How to Cite
de Oliveira, F. L., Vasconcellos, B. O., de Morais, T. S., Nascimento, M. B., Teles, R., da Costa Nery, J. A., Miranda, M. J. S. and Azulay-Abulafia, L. (2013), Netherton’s syndrome and lepromatous leprosy: a mere coincidence?. International Journal of Dermatology, 52: 186–190. doi: 10.1111/j.1365-4632.2012.05620.x
- Issue published online: 24 JAN 2013
- Article first published online: 24 JAN 2013
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.