Leiner's disease
Leiner's Disease
Leiner's disease (pronounced: /ˈlaɪnərz diːˈziːz/) is a rare, severe skin disorder that primarily affects infants and young children.
Etymology
The condition is named after the Hungarian pediatrician, Carl Leiner, who first described the disease in 1926.
Definition
Leiner's disease, also known as Erythroderma desquamativum, is characterized by widespread erythroderma, severe diarrhea, recurrent infections, and failure to thrive. It is often associated with a deficiency in the complement system, specifically the fifth component (C5).
Symptoms
The primary symptom of Leiner's disease is a severe, generalized erythroderma that usually begins in the first weeks of life. Other symptoms may include diarrhea, failure to thrive, recurrent infections, and in severe cases, sepsis.
Causes
The exact cause of Leiner's disease is unknown. However, it is often associated with a deficiency in the complement system, specifically the fifth component (C5). This deficiency can lead to an increased susceptibility to infections, which can trigger the onset of the disease.
Treatment
Treatment for Leiner's disease primarily involves managing the symptoms and preventing infections. This may include the use of antibiotics, steroids, and immunoglobulin therapy. In severe cases, hospitalization may be required.
Prognosis
The prognosis for Leiner's disease varies. With early diagnosis and appropriate treatment, many children can lead normal lives. However, in severe cases, the disease can be life-threatening.
See Also
External links
- Medical encyclopedia article on Leiner's disease
- Wikipedia's article - Leiner's disease
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