3.2 | Case 2
6 years later, the mother became pregnant again. At 4 weeks of
pregnancy, serologic tests found strongly positive SSA (Ro) and SSB (La)
antibodies, a reactive ANA of
>1:1000 with multiple nuclear dots, and an elevated ESR of
90mm/h (normal < 10mm/h). Due to the mother’s active
condition, she was treated with prednisone 10mg/d, hydroxychloroquine
400mg/d, and calcium carbonate 600mg/d. and her doctor suggested that
close monitoring was necessary throughout the perinatal period. However
she didn’ t strictly implement.
A
male infant
(patient 2) was born at 39
weeks’ gestation by performing a
caesarean section because of fetal macrosomia,
with a birthweight of
4500g.
One week after birth, the child
gradually developed a rash dominated by
erythema and papules (Fig. 2a),
with different sizes and partially
fused into patches. The skin lesions were limited, mainly on the head
and face. This was quite different from
the rash of his
sister,
which were mainly characterized by scaly red patches, with a wide range
of skin lesions, including the head, face, trunk, and limbs. The test
found both the SSA (Ro) and SSB (La) were positive, ANA was reactive
1:100 with multiple nuclear dots.
Skin
biopsy showed
vacuolar change of
epidermal basal cells, perivascular
and periadnexal mononuclear infiltrate in the dermis (Fig. 2b). A
diagnosis of NLE was made as his
sister.
The rash start to partially subside
from 2.5 months,
and the rash resolved after 5.5
months without specific therapy, which was very similar to his sister.
At present, both siblings are still in
follow-up.
The typical cutaneous manifestations of NLE include erythematous annular
plaques. Lesions in the periorbital
region often result in the characteristic appearance of an “eye mask”
or a “raccoon-like”. In our cases, the
skin lesions of patient 1
showed
typical annular plaques and patient
2 showed untypical rare red papules, which is
very interesting.
Consent for publication of this case report and accompanying images was
obtained from the patient’s parents after they were fully informed and
had provided written authorization.