Congenital and neonatal lupus erythematosus: two case reports.

Neonatal lupus erythematosus is an autoimmune disease produced by the passage of maternal antinuclear antibodies and extractable nuclear antigen antibodies through the placenta. At the moment of the diagnosis, the mothers are asymptomatic in 40 to 60% of cases. The most common manifestations are cutaneous lesions and congenital heart block. The cutaneous findings are variable and usually begin within the first weeks or months of life. Congenital lupus erythematosus is a congenital variant of neonatal lupus erythematosus. We present one case of congenital lupus erythematosus and one case of neonatal lupus erythematous, showing the variability of this disease.

Congenital and neonatal lupus erythematosus: two case reports / Lúpus eritematoso congênito e neonatal: relato de dois casos

Neonatal lupus erythematosus is an autoimmune disease produced by the passage of maternal antinuclear antibodies and extractable nuclear antigen antibodies through the placenta. At the moment of the diagnosis, the mothers are asymptomatic in 40 to 60% of cases. The most common manifestations are cutaneous lesions and congenital heart block. The cutaneous findings are variable and usually begin within the first weeks or months of life. Congenital lupus erythematosus is a congenital variant of neonatal lupus erythematosus. We present one case of congenital lupus erythematosus and one case of neonatal lupus erythematous, showing the variability of this disease.

Lúpus eritematoso neonatal é uma doença auto-imune produzida pela passagem de anticorpos maternos antinucleares e anticorpos contra antígenos extraíveis nucleares através da placenta. No momento do diagnóstico, as mães são assintomáticas em 40 a 60% dos casos. As manifestações mais comuns são lesões cutâneas e bloqueio cardíaco congênito. Os achados cutâneos são variáveis e geralmente começam nas primeiras semanas ou meses de vida. Lúpus eritematoso congênito é uma variante do lúpus eritematoso neonatal. Apresentaremos um caso de lúpus eritematoso congênito e um caso de lúpus eritematoso neonatal, mostrando a variabilidade da doença.

Neonatal lupus erythematosus: a report of four cases.

Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10% of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95%), La and, less frequently, U1-ribonucleoprotein (U1-RNP). The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress.

Lupus eritematoso neonatal: reporte de cuatro casos / Neonatal lupus erythematosus: a report of four cases

El lupus eritematoso neonatal es una enfermedad poco frecuente, caracterizada clínica mente por alteraciones cutáneas semejantes al lupus subagudo o discoide y/o bloqueo cardíaco congénito. Generalmente, cuando los pacientes presentan manifestaciones cutáneas, no tienen anormalidades cardiológicas y viceversa, aunque en un 10 por ciento de los casos ambas manifestaciones pueden coexistir. Puede acompañarse también de alteraciones hematológicas, hepáticas y neurológicas. Es causado por el pasaje trasplacentario de anticuerpos maternos anti Ro (95 por ciento), anti La y menos frecuentemente anti U1RNP. Presentamos cuatro pacientes con hallazgos clínicos, histopatológicos e inmunológicos compatibles con lupus eritematoso neonatal, su tratamiento y evolución.

Neonatal lupus erythematosus is a very rare disease, clinically characterized by skin lesions that resemble those of subacute or discoid lupus erythematosus and/or congenital heart block. Generally, when patients have skin manifestations, they have no cardiac defects and vice-versa; however, in 10 percent of cases these manifestations may coexist. Other findings may include hematologic, hepatic and neurological abnormalities. This condition is caused by the transplacental passage of maternal autoantibodies against Ro (95 percent), La and, less frequently, U1-ribonucleoprotein (U1-RNP). The present case report describes four patients with clinical, histopathological and immunological findings compatible with neonatal lupus erythematosus, their treatment and progress.

Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry.

OBJECTIVE: To extend the information base on cutaneous manifestations of neonatal lupus erythematosus (NLE) with regard to maternal disease, sex of child, onset, localization, influence of UV light, prognosis, and recurrence rates in subsequent pregnancies. METHODS: Review of records from the Research Registry for Neonatal Lupus. RESULTS: The cohort includes 47 mothers (83% white) whose sera contain anti-SSA/Ro, anti-SSB/La, and/or anti-U1-ribonucleoprotein antibodies and their 57 infants (20 boys and 37 girls) diagnosed with cutaneous NLE (absent heart disease) between 1981 and 1997. At detection of the child's rash, 13 mothers were asymptomatic, 11 had an undifferentiated autoimmune syndrome (UAS), 9 had systemic lupus erythematosus (SLE), 7 Sjögren's syndrome (SS), 6 SLE/SS, and 1 rheumatoid arthritis/SS; 20 reported photosensitivity. Within 5 years, 7 asymptomatic mothers experienced disease progression: 1 developed photosensitivity, 2 SLE, 3 SS, 1 SLE/SS; in 2 mothers UAS progressed to SLE; and 2 mothers with SS developed SLE. The infant's rash often followed UV light exposure; mean age at detection was 6 weeks, and mean duration was 17 weeks. All had facial involvement (periorbital region most common) followed by the scalp, trunk, extremities, neck, and intertriginous areas. In 37, the rash resolved without sequelae, 43% of which were untreated. A quarter had residual sequelae that included telangiectasia and dyspigmentation. One child developed Hashimoto's thyroiditis, and 2 developed systemic-onset juvenile rheumatoid arthritis. Of 20 subsequent births, 7 children were healthy, 2 had congenital heart block (CHB) only, 4 CHB and skin rash, and 7 skin rash only. CONCLUSIONS: Future pregnancies should be monitored by serial echocardiograms, given the substantial risk for heart block. Affected children should be observed for later development of a rheumatic disease.

Neonatal lupus erythematosus.

The patient is a newborn girl, born at term by cesarian section, weighing 3,550 g, and measuring 50 cm in length. According to her mother, she had been presenting erythematous spots involving the scalp, face, and trunk since birth that had increased in size with time. The mother denied any other changes and reported normal growth and development. Physical examination at 2 months of age revealed an infant in good general condition, hydrated, with no fever and no abnormalities detected on careful physical examination. The dermatologic examination revealed numerous circumscribed erythematous-brownish flat maculae with sharp borders and irregular contours, with no follicular hyperkeratosis, but with telangiectasias and areas of atrophy. They were lenticular and nummular, especially on the face (peri-orbital heliotropic lesions), and appeared as plaques on the trunk, with a tendency to confluence. The lesions also involved the scalp, neck, and extremities (Fig. 1). Histopathologic examination revealed an atrophic epidermis with focal areas of hydropic degeneration of the basal layer. A discrete perivascular and periannexal lymphohistiocytic infiltrate was observed in the superficial dermis, with melaninophages, frequent extravasation of red blood cells, angiectasia, and edema (Fig. 2). Serologic tests were reactive for antinuclear factor (ANF) (titer 1:100) of the speckled pattern, the presence of anti-Ro antibodies, and absence of anti-RNA and anti-Sm antibodies (Table 1). Blood counts and electrocardiogram were normal (Table 1). HLA typing showed positivity for DR-3 (Table 2). At 5 months of age the patient already showed a marked improvement of the skin lesions with only some areas of discrete pigmentation, a few atrophic areas, and rare telangiectasis (Fig. 3). The serologic tests (ANF, anti-Ro) had become nonreactive and the anti-RNA and anti-Sm tests continued to be negative. Examination of the mother revealed an asymptomatic 25-year-old woman reporting no manifestations suggestive of lupus. General and special physical examination revealed no abnormalities. Dermatologic examination showed no active or residual lesions of discoid or systemic lupus erythematosus. The pregnancy had been uneventful. Histopathologic examination of the girl's skin revealed the epidermis without obvious changes, minimal edema in the dermis, and a discrete perivascular inflammatory mononuclear cell infiltrate. Direct immunofluorescence of normal skin not exposed to the sun was negative. Blood counts revealed mild anemia and a tendency to leukopenia and thrombocytopenia (Table 1). Serologic tests showed reactive ANF (titers 1:400 and 1:800, speckled pattern), the presence of anti-Ro antibodies, and the absense of anti-RNA and anti-Sm antibodies (Table 1). HLA-typing revealed positivity for DR-3 (Table 2).

Lupus eritematoso sistemico e gestacao / Sistemic lupus erythemathosus and pregnancy

O lupus eritematoso sistemico (LES) e uma doenca auto-imune que afeta mulheres durante a idade reprodutora. Os autores discutem os efeitos da gestacao no lupus, sendo que as condicoes ideais para a paciente lupica engravidar seriam: dois anos do diagnostico da doenca, no minimo apos 6 meses de remissao da doenca e nivel de creatinina serica previa a gestacao < 1,6mg/dl. Fatores de mau prognostico durante a gestacao seriam: atividade da doenca na concepcao e diagnostico de lupus durante a gestacao. As complicacoes do LES na gestacao a caracterizam como gestacao de altissimo risco: perdas fetais de 29 por cento, prematuridade de 15 a 20 por cento e crescimento intra-uterino retardado de 30 por cento dos casos. A presenca da sindrome antifosfolipide e de grande risco para a gestacao, elevando as perdas fetais ate 90 por cento. O tratamento mais efetivo para esta complicacao ainda nao esta estabelecido. A sindrome de lupus neonatal esta associada com a presenca do anticorpo anti-RO/anti-La e caracteriza-se por um quadro cutaneo transitorio sem sequelas e pela presenca de BAV congenito que e definitivo, porem infrequente (1,5 por cento). Os autores sugerem ainda uma conduta pratica para o seguimento pre-natal, que deve ser precoce e permitir um amplo esclarecimento dos riscos para a paciente.