title: Neonatal lupus erythematosus complicated by mucocutaneous and visceral hemangiomas.
abstract: Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune syndrome resulting from transplacental passage of maternal anti-Ro/SSA and/or anti-La/SSB antibodies to the fetus. Characteristic manifestations of NLE include transient dermatitis, hepatic and hematologic abnormalities and congenital heart block. Skin lesions in NLE resemble subacute cutaneous lupus erythematosus and typically consist of annular, erythematous, scaly plaques. Telangiectasias, vascular abnormalities resulting from dilation of superficial dermal vessels, may also affect the skin in a minority of patients. The etiology of telangiectasias in NLE is unknown, but disordered angiogenesis likely plays a role. Hemangiomas are a common disorder of angiogenesis frequently encountered in infancy. There have been no reported cases of neonatal lupus associated with the development of hemangiomas. We present a case of an infant diagnosed with NLE after manifesting classic dermatitis, hepatic and hematologic abnormalities who later developed mucocutaneous and visceral hemangiomas. We further postulate that disordered angiogenesis, possibly dysregulated production of vascular endothelial growth factor, may play a primary role in the development of these cutaneous vascular lesions in NLE.
authors.affiliation: Division of Rheumatology, Children's Hospital of Pittsburgh, Pittsburgh, PA 15213, USA.
authors.forename: S J
authors.initials: SJ
authors.lastname: Spalding
authors.forename: T
authors.initials: T
authors.lastname: Hennon
authors.forename: J
authors.initials: J
authors.lastname: Dohar
authors.forename: T
authors.initials: T
authors.lastname: Arkachaisri
mesh_terms: D000328:Adult; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D000974:Antibodies, Antinuclear / Q000276:immunology; D005260:Female; D018324:Hemangioma, Capillary / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D008107:Liver Diseases / Q000276:immunology / Q000473:pathology; D008180:Lupus Erythematosus, Systemic / Q000150:complications; D011239:Prednisolone / Q000627:therapeutic use; D011247:Pregnancy; D011248:Pregnancy Complications / Q000276:immunology*; D012261:Ribonucleoproteins / Q000276:immunology; D013684:Telangiectasis / Q000209:etiology*; D000099207:SS-A Antigen
chemical_list: D000893:Anti-Inflammatory Agents; D000974:Antibodies, Antinuclear; D011239:Prednisolone; D012261:Ribonucleoproteins; D000099207:SS-A Antigen; C035357:SS-B antibodies