engleski

Neonatal lupus erythematosus in non-identical twins

Objectives: Neonatal lupus erythematosus (NLE) is a rare syndrome caused by passive transplacental transport to foetal circulation of maternal IgG autoantibodies of SS-A or SS-B type, directed against auto-antigens and causing clinical manifestations in neonate. The incidence of NLE is approximately 2% in offspring of SS-A/SS-B positive mothers. The clinical features include rash, hepatobiliary disease and cytopenias, all of which disappear with the clearance of maternal autoantibodies. Macular or papular annular lesions with fine scaly borders and central clearing usually appear on the face and scalp. Periorbital lesions give rise to “raccoon-like” appearance. Close monitoring for atrioventricular (AV) block is mandatory in neonatal period. Method: A 4-week old female born from twin pregnancy of asymptomatic mother presented with widespread annular scaly erythematous rash. Skin biopsy was performed. The histology was non-specific with reactive changes in the epidermis and mild to moderate monocyte and lymphocyte infiltrate in dermis and subcutaneous fat. PAS staining and immunohistochemistry was negative for CD20, S-100, CD1a and CD117. The rash progressed after sun exposure and at the age of 8 weeks, patient's twin brother developed similar lesions. Results: Clinical diagnosis of NLE was confirmed by positive ANA, SS-A, SS-B, and Ro52 antigen in asymptomatic mother and both children. Children were assessed by multidisciplinary team and the results showed no systemic involvement. At the age of 7 months skin lesions completely resolved in both twins. Discussion: NLE is rare condition presenting with rash that might be misdiagnosed as eczema or infection with a risk to overlook potentially life threatening AV block. Our twins case highlighted that NLE could be easily missed and should be considered in neonates with non- specific annular rashes born from asymptomatic mothers. The correct diagnosis was made and confirmed by blood tests based on clinical features of the twin brother.

Non-identical twins, Neonatal lupus, Annular rash

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