Neonatal lupus erythematosus in an infant with Turner syndrome.
Neonatal lupus erythematosus (NLE) is characterized by transient, annular cutaneous lesions, congenital heart block, and a variety of systemic or hematologic abnormalities. We describe a white infant girl with onset of skin lesions on the face and scalp at 4 days of age. At age 4 weeks she had generalized, erythematous, scaly, annular skin lesions that underwent spontaneous regression at age 5 months. Her mother had no cutaneous or other lesions, but complement examinations revealed the presence of anti-Ro(SSA) and anti-La(SSB) antibodies, and absence of anti-Sm and anti-RNP antibodies. Karyotyping revealed Turner syndrome (TS) with 45,XO sex chromosome constitution. Ro(SSA) and La(SSB) antibodies were found, and direct immunofluorescence testing on healthy skin was positive. At age 5 months, follow-up immunologic examination of the infant had normal results but the mother still had anti-Ro(SSA) and anti-La(SSB) antibodies. We believe that this is the first reported case of NLE in association with TS.
Ruas
E
E
Dermatology Clinic, Hospitais da Universidade de Coimbra, Portugal.
Moreno
A
A
Tellechea
O
O
Rocha
G
G
Baptista
A P
AP
D000974:Antibodies, Antinuclear / Q000032:analysis; D001324:Autoantigens / Q000032:analysis; D005148:Facial Dermatoses / Q000150:complications / Q000473:pathology; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D005500:Follow-Up Studies; D006327:Heart Block / Q000151:congenital; D006801:Humans; D007231:Infant, Newborn; D007621:Karyotyping; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications* / Q000473:pathology; D012075:Remission, Spontaneous; D017411:Ribonucleoproteins, Small Nuclear / Q000276:immunology; D012536:Scalp Dermatoses / Q000150:complications / Q000473:pathology; D014424:Turner Syndrome / Q000150:complications*; D014960:X Chromosome; D055517:snRNP Core Proteins
D000974:Antibodies, Antinuclear; D001324:Autoantigens; D017411:Ribonucleoproteins, Small Nuclear; C035356:SS-A antibodies; C035357:SS-B antibodies; D055517:snRNP Core Proteins