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


CONCLUSIONS
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.
Section for Sexually Transmitted Diseases and Leprosy, Professor RD Azulay Institute of Dermatology, Hospital Santa Casa da Misericórdia, Rio de Janeiro, Brazil.
Felipe Ladeira
FL
de Oliveira
Bruna Obeica
BO
Vasconcellos
Thais Schiavo
TS
de Morais
Mayara Brito
MB
Nascimento
Renata
R
Teles
José Augusto
JA
da Costa Nery
Marcos José Sumrell
MJ
Miranda
Luna
L
Azulay-Abulafia
D000328:Adult; D001938:Brazil; D005808:Genes, Recessive; D020022:Genetic Predisposition to Disease; D006801:Humans; D015440:Leprosy, Lepromatous / Q000150:complications* / Q000235:genetics*; D008297:Male; D056770:Netherton Syndrome / Q000150:complications* / Q000235:genetics*; D053491:Proteinase Inhibitory Proteins, Secretory / Q000235:genetics*; D000074163:Serine Peptidase Inhibitor Kazal-Type 5
D053491:Proteinase Inhibitory Proteins, Secretory; C120650:SPINK5 protein, human; D000074163:Serine Peptidase Inhibitor Kazal-Type 5