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.