The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus.
BACKGROUND
Cutaneous neonatal lupus erythematosus (NLE) is an uncommon disease described mainly through isolated case reports.


OBJECTIVE
Our purpose was to examine the cutaneous spectrum, clinical associations, and course of disease in babies with anti-Ro-positive NLE.


METHODS
This is a retrospective case series evaluation of newborns with anti-Ro-positive NLE seen at a single ambulatory care university center over a 20-year period. Cases were drawn from a population of 3.2 million. Follow-up was at least 3 years.


RESULTS
Four boys and 14 girls were included in our evaluation. Distribution of skin lesions in 18 babies was as follows: face, 17; periorbital "owl-eye" or "eye mask" facial rash, 14; scalp, 15; arms and legs, 13; trunk and groin, 6. Crusted lesions were predominant in 3. Photosensitivity was seen in 12, and features of cutis marmorata telangiectasia congenita were observed in 4. In 17 neonatal lupus was not suspected until the dermatology consultation. Noncutaneous manifestations included thrombocytopenia in 4, cholestatic hepatitis in 3, and congenital heart block in 3. Four patients had residual telangiectasia that persisted for 3 or more years but eventually cleared in 2 patients. Three babies had dyspigmentation that spontaneously cleared within 22 months. None had atrophy or scarring.


CONCLUSIONS
Periorbital, scalp, and extremity lesions are common in cutaneous NLE. Crusted lesions predominated in male infants. In children selected by cutaneous involvement, thrombocytopenia and hepatic disease were present as frequently as cardiac disease and occurred more frequently in male babies with crusted skin lesions. Children with cutaneous NLE should be evaluated for hematologic and hepatic as well as cardiac involvement.
D000974:Antibodies, Antinuclear / Q000032:analysis*; D002779:Cholestasis / Q000150:complications; D005141:Eyelid Diseases / Q000151:congenital / Q000276:immunology; D005148:Facial Dermatoses / Q000151:congenital / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006327:Heart Block / Q000151:congenital; D006505:Hepatitis / Q000150:complications; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007868:Leg Dermatoses / Q000151:congenital / Q000276:immunology; D008178:Lupus Erythematosus, Cutaneous / Q000151:congenital* / Q000276:immunology; D008297:Male; D010787:Photosensitivity Disorders / Q000151:congenital; D010859:Pigmentation Disorders / Q000151:congenital; D012189:Retrospective Studies; D012536:Scalp Dermatoses / Q000151:congenital / Q000276:immunology; D012737:Sex Factors; D013684:Telangiectasis / Q000151:congenital; D013921:Thrombocytopenia / Q000150:complications