Leiner's disease (LD) is a rare and serious syndrome of infantile
erythroderma of severe and progressive generalized seborrheic-like
dermatitis, recalcitrant
diarrhea, malabsorption and wasting, and recurrent local and systemic
infections. The purpose of this study is to provide an updated review on management with a summarized review of available peer-reviewed articles on LD. The mechanisms underlying this disease process remain unclear. The diagnosis includes demonstration of deficient opsonic activity along with the clinical tetrad of
erythroderma, persistent gastrointestinal disturbance, superimposed bacterial or candidal
infection, and marked wasting. An important correlation between LD and defective yeast and Staphylococcus aureus opsonization has been established. For the familial form of LD, an association of either
complement three deficiency or
complement five dysfunction has been made. LD should be distinguished from other types of infantile
erythroderma, including
Omenn syndrome. Treatment includes fluid and nutrition replacement,
antibiotics to control infection, and fresh-frozen plasma
therapy. The prognosis is unclear; it depends on treatment. LD is a life-threatening condition that requires prompt identification and hospitalization. Affected infants who receive vigorous treatment not only have the prospect of surviving, but also generally lead a normal life after infancy.