Leiner's disease
(Redirected from Erythroderma desquamativum)
Leiner's disease is a rare dermatological condition characterized by severe seborrheic dermatitis and recurrent infections. It primarily affects infants and is often associated with an underlying immunodeficiency. The disease is named after the Austrian dermatologist Karl Leiner, who first described it in the early 20th century.
Clinical Features[edit | edit source]
Leiner's disease typically presents in the first few months of life. The main clinical features include:
- Severe seborrheic dermatitis: This manifests as erythematous, scaly lesions on the scalp, face, and diaper area.
- Recurrent infections: Infants with Leiner's disease are prone to bacterial, viral, and fungal infections due to an underlying immunodeficiency.
- Failure to thrive: Affected infants may exhibit poor weight gain and growth.
Pathophysiology[edit | edit source]
The exact cause of Leiner's disease is not well understood. However, it is believed to be associated with a defect in the complement system, particularly involving the C5 component. This defect leads to an impaired immune response, making the infant susceptible to infections and severe dermatitis.
Diagnosis[edit | edit source]
Diagnosis of Leiner's disease is primarily clinical, based on the characteristic skin lesions and recurrent infections. Laboratory tests may reveal abnormalities in the complement system, and skin biopsy can show nonspecific inflammatory changes.
Treatment[edit | edit source]
Management of Leiner's disease involves:
- Topical treatments: Use of emollients and anti-inflammatory agents to manage seborrheic dermatitis.
- Antibiotics and antifungals: To treat and prevent infections.
- Nutritional support: Ensuring adequate nutrition to support growth and development.
Prognosis[edit | edit source]
The prognosis for infants with Leiner's disease varies. With appropriate treatment, many infants experience improvement in symptoms. However, the underlying immunodeficiency may persist, requiring ongoing medical care.
See Also[edit | edit source]
References[edit | edit source]
External Links[edit | edit source]
| Leiner's disease Resources | |
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