Neonatal lupus erythematosus
| Neonatal lupus erythematosus | |
|---|---|
| Synonyms | NLE |
| Pronounce | N/A |
| Specialty | N/A |
| Symptoms | Rash, congenital heart block, liver dysfunction, hematological abnormalities |
| Complications | Congenital heart block |
| Onset | Neonatal |
| Duration | Varies, rash typically resolves within 6 months |
| Types | N/A |
| Causes | Transplacental passage of maternal autoantibodies |
| Risks | Maternal systemic lupus erythematosus, Sjogren's syndrome |
| Diagnosis | Clinical evaluation, serology for maternal autoantibodies |
| Differential diagnosis | Erythema toxicum neonatorum, seborrheic dermatitis, atopic dermatitis |
| Prevention | N/A |
| Treatment | Supportive care, corticosteroids for severe cases |
| Medication | N/A |
| Prognosis | Generally good, except for cases with congenital heart block |
| Frequency | Rare |
| Deaths | N/A |
Neonatal lupus erythematosus (NLE) is a rare autoimmune disease that affects newborns. It is caused by the transplacental passage of maternal autoantibodies to the fetus. The most common clinical manifestations are skin rash, heart block, and hematological abnormalities.
Etiology
NLE is caused by the passage of maternal autoantibodies, specifically anti-Ro/SSA and anti-La/SSB, across the placenta to the fetus. These antibodies are found in mothers with autoimmune diseases such as SLE and Sjögren's syndrome. However, some mothers of infants with NLE do not have any symptoms of these diseases.
Clinical Manifestations
The most common clinical manifestations of NLE are:
- Skin rash: This is often the first sign of NLE. It usually appears within the first few weeks of life and resolves within six months. The rash is typically annular and erythematous, and is most commonly found on the face and scalp.
- Heart block: This is a serious complication of NLE and can lead to death. It is usually detected in utero and is irreversible.
- Hematological abnormalities: These can include thrombocytopenia, neutropenia, and anemia.
Diagnosis
The diagnosis of NLE is based on clinical findings and the presence of maternal autoantibodies. Skin biopsy can be used to confirm the diagnosis.
Treatment
There is no specific treatment for NLE. Management is supportive and includes sun protection for the skin rash and pacemaker implantation for heart block.
Prognosis
The prognosis for NLE is generally good. The skin rash and hematological abnormalities usually resolve within six months. However, heart block is a serious complication and can lead to death.
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