What is Leiner disease?
Leiner disease occurs in infants and is characterised by severe generalised seborrhoeic dermatitis, recurrent diarrhoea, recurrent skin and internal infections, and failure to thrive. It is also known as ‘erythroderma desquamativum’. Leiner disease may be present at birth but more commonly develops within the first few months of life. It appears to be more common in females than males and in breast-fed infants.
What causes Leiner disease?
The precise cause of Leiner disease remains unknown but it is known that a defect in the body's complement system has a major role to play in its development. The complement system is a vital part of the body's immune system, and in Leiner disease an inherited dysfunction or deficiency in the C5 component of complement alongside other factors have been implicated. Other immune deficiencies may present in an identical fashion in infancy.
What are the signs and symptoms?
The condition usually starts off as a scaly rash on the scalp, face or napkin area. Very rapidly it spreads to other parts of the body. The affected area is bright red and may look swollen. Infants appear uncomfortable but do not itch. Other symptoms include recurrent diarrhoea, infant not thriving or gaining weight, and local skin infections. There is also a risk of developing more severe infections that may lead to pneumonia, meningitis and septicaemia.
What treatment is available?
Initially affected babies may need to be hospitalised to manage fluid and heat loss. Bland emollients may be used to treat the rash. Providing adequate nutrition is also an essential part of treatment.
Biotin, a water-soluble vitamin that is found naturally in foods such as liver, kidney, meat, milk, egg yolks and vegetables, appears to be useful in treating Leiner disease.
Book: Textbook of Dermatology. Ed Rook A, Wilkinson DS, Ebling FJB, Champion RH, Burton JL. Fourth edition. Blackwell Scientific Publications.
On DermNet NZ:
- Complement deficiencies – Medscape Reference
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