Neonatal Lupus Erythematosus

Extracutaneous Findings Beyond Heart

Block
One percent of NLE patients develop
hepatic disease.
o Differential diagnosis:
Metabolic, infectious, and
inherited anatomic
(extrahepatic biliary atresia)
conditions must be ruled out.
Thrombocytopenia or neutropenia is
observed in about 10% of cases. This
resolves in 2 to 3 weeks, but can be
severe.
Central nervous system disease has
been rarely reported in NLE.

Diagnostic Workup
The diagnosis should be based on the
maternal history of anti-Ro/La
antibodies, a previous child with NLE, or
autoimmune disease, especially
photosensitivity, dry eyes, and dry
mouth.
The clinical features of early onset of
scaly plaques on the face, scalp, and
extremities, especially in a periorbital
distribution, must prompt consideration
of NLE. Obviously, infants with
complete heart block likely have NLE.
Maternal history and review of
symptoms must be done.
If the diagnosis is suspected, a maternal
ANA and anti-Ro and anti-La antibodies
are reasonable. The baby's sera may be
tested for anti-Ro and La as well.
A complete blood count, liver function
tests, and an ECG should be done to
rule out extracutaneous involvement.

Laboratory Studies
NLE is related to the anti-Ro (SS-A)
antibody in more than 90% of patients.
Occasionally, patients only have anti-La
(SS-B) or anti-U1RNP antibodies.
Test for cytopenia (CBC count) and liver
enzyme elevations.
Children in whom systemic lupus
erythematosus (SLE) is suspected
should undergo a serologic evaluation,
including antinuclear antibody (ANA),
anti-dsDNA, anti-Sm, anti-RNP, anti-Ro
(SS-A), and anti-La (SS-B), as well as
measurement of complement levels.
Also test for other organ involvement,
including a CBC count and tests of renal
function.
Other Tests
Electrocardiography reveals the degree of heart
block
Procedures
Skin biopsy is useful in patients with either
neonatal lupus erythematosus or cutaneous
lesions of lupus erythematosus during
childhood.
Histologic Findings
Skin biopsy findings reveal interface
dermatitis.
Epidermal atrophy may be found.
Inflammatory infiltrate may be so
intense that bulla formation may
develop histologically.

Treatment

Medical Care
Neonatal lupus erythematosus (NLE)
that affects the skin, blood, spleen, or
liver is usually self-limited and resolves
without intervention within 2-6 months.
In severe cases, neonatal lupus
erythematosus that affects the heart
may result in cardiac failure and death.
A pacemaker is often necessary. Treat
skin conditions with sunscreen, topical
corticosteroids, and antimalarial agents.
Surgical Care
No specific surgical care is indicated
Pacemaker placement may be needed.
Consultations
Dermatologist
Rheumatologist
Nephrologist
Neurologist
Immunologist
Hematologist
Activity
Limit activity only if the disease is active
and base restrictions on the patient's
abilities.
Patients with skin disease should
restrict their sun exposure.

Medication
NLE does not require specific therapy.
Sunscreens are also useful in the treatment of
cutaneous lupus. Sunscreens do not block all
wavelengths of light. In adult studies, the
wavelengths of light responsible for induction
of cutaneous lupus erythematosus are within
the UV-B and UV-A range.
Topical corticosteroids
Use to control cutaneous lesions. Select a
specific agent based on treatment site and type
of lesion. Facial skin is more prone to atrophy
than skin of the scalp or hands; use a weaker
agent on the face. Thick lesions may require
more potent agents.
Immunosuppressive agents
Patients with immune dysregulation and
autoimmunity often benefit from
immunosuppression. These drugs are useful in
patients with skin disease that is unresponsive
to topical agents and in patients with arthritis
that does not respond to NSAIDs. These drugs
are not needed in neonatal lupus
erythematosus but may be used in children with
skin or joint disease of systemic lupus
erythematosus.


Follow Up
Patients with NLE of the skin do not
require monitoring after lesions resolve.
They may be more prone to develop
lupus erythematosus later in life, but
this reflects their genetic
predisposition, not that they had
neonatal lupus erythematosus. Their
nonaffected siblings are also at risk for
development of systemic lupus
erythematosus.
Regular monitoring to assess cardiac
function and the need for a pacemaker.
Prevention
Mothers of neonates with NLE,
particularly neonates with CHB, have a
2-fold to 3-fold increased risk of
subsequent affected neonates. An
estimated 25% of subsequent
pregnancies are affected. Therefore,
carefully monitor subsequent
pregnancies, particularly at 18-24
weeks gestation.

Prognosis
Generally, skin disease is self-limited.
Hepatosplenomegaly is self-limited, although
death due to hepatitis may occur.
Cytopenia is self-limited; however, if severe
thrombocytopenia is present, bleeding can
affect the prognosis.
Cardiac involvement is associated with a poor
outcome in many patients, including some who
receive pacemakers.