Neonatal Lupus Erythematosus Presenting with "Eye Mask" Facial Rash and Telangiectasia / 대한피부과학회지

Neonatal lupus erythematosus (NLE) is an autoimmune disease associated with the maternal anti-SSA/Ro and anti-SSB/La antibodies. NLE is characterized by cutaneous erythema, congenital heart block (CHB), hepatic dysfunction and hematological abnormalities. The characteristic cutaneous lesion is an annular or polycyclic erythematous patch or plaque. Individual annular lesions are often about 1 cm in diameter. We report a case of NLE in a 7-day-old female infant, born of a clinically asymptomatic mother, presenting "eye mask" shape rash and telangiectasia on her face. Periorbital rash with "eye mask" appearance presents in a few of NLE cases, and this cutaneous sign has been emphasized as a diagnostic clue to NLE. Her serological tests revealed high titers of anti-SSA/Ro, anti-SSB/La and antinuclear antibody. Based on these clinical and serological findings, the patient was diagnosed as NLE with characteristic periorbital skin findings.

Cutaneous neonatal lupus erythematosus with unusual features.

A three month-old boy was brought by his mother with complaints of multiple reddish lesions on his trunk and face since birth. The patient had erythematous annular plaques with scaling on his extremities, palms and soles with periorbital erythema and edema giving the characteristic "eye mask" or "owl's eye" appearance. His mother did not have history of any illness. Hemogram, liver and renal function tests were within normal limits. A skin biopsy was suggestive of subacute cutaneous lupus erythematosus. Immunological work-up was positive for antinuclear antibodies (ANA) (1:40) with anti-Ro titers of 3.4 and 3.47 (>1.1 = clinically significant titre) in the mother and child respectively, although negative for anti-La antibodies. The child's electrocardiogram and 2D echocardiography were normal. We are presenting a case of anti-Ro-positive cutaneous lupus erythematosus with an uncommon skin manifestation.