The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus

• Published in: Journal of the American Academy of Dermatology Vol. 40; no. 5; pp. 675 - 681
• Main Authors: Weston, William L., Morelli, Joseph G., Lee, Lela A.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 01.05.1999; Elsevier
• Subjects: Antibodies, Antinuclear - analysis; Biological and medical sciences; Cholestasis - complications; Eyelid Diseases - congenital; Eyelid Diseases - immunology; Facial Dermatoses - congenital; Facial Dermatoses - immunology; Female; Follow-Up Studies; Heart Block - congenital; Hepatitis - complications; Humans; Infant; Infant, Newborn; Leg Dermatoses - congenital; Leg Dermatoses - immunology; Lupus Erythematosus, Cutaneous - congenital; Lupus Erythematosus, Cutaneous - immunology; Male; Medical sciences; Photosensitivity Disorders - congenital; Pigmentation Disorders - congenital; Retrospective Studies; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Scalp Dermatoses - congenital; Scalp Dermatoses - immunology; Sex Factors; Telangiectasis - congenital; Thrombocytopenia - complications; Human; Immunopathology; Connective tissue disease; Symptomatology; Skin disease; Chronic; Newborn; Systemic disease; Autoimmune disease; Lupus erythematosus
• ISSN: 0190-9622; 1097-6787
• DOI: 10.1016/S0190-9622(99)70146-5

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. Conclusion: 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.(J Am Acad Dermatol 1999;40:675-81.)