Neonatal Lupus Associated with Dyslipidemia Case Report.pdfof dyslipoproteinemia characterized by...
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A male patient, three months old, born at term gestation without com- plications, was taken by his mother for medical evaluation due to a two- month evolution, large, bilateral, ery- thematous, fine-scaling mask-like plaque on the face, which was wors- ened by sun exposure (Figs. 1,2). After long evaluation, the possibility of Neonatal Lupus Syndrome was suspected and ANA of the patient was collected. Serum sample was ini- tially rejected due to its unexpectedly heavy hyperlipidemic nature, which persisted in subsequent samples. Hemogram and Lypidogram tests were collected, presenting the fol- lowing results: eight percent eosinophilia, 70 percent lymphocytosis, and triglyceride levels of 976mg/dL. ANA was ultimately tested and demonstrated posi- tive in 1:640. Skin biopsy confirmed the diagnosis of Lupus. Electrocardiogram has shown no impairment. Parallel mother evaluation demonstrated speckled- patterned ANA anti-Hep 1:2560 and anti-Ro 1:3200, despite lack of rheumatologic disease diagnosis and symptoms. No nephrologic impairment was revealed during evaluation of the child. Skin lesions remitted after four months of treatment. Triglyceride levels reached normality after eight months. The patient was subject- ed to evaluation every three months during his first year of age and semiannually until four years old and remained asymptomatic during monitoring. Discussion The neonatal lupus syndrome (NLS) is a rare disease characterized by an autoimmune process associated with the presence of auto antibodies in maternal fetal circulation against proteins SSA/Ro and SSB/La. Its clinical manifestations include cutaneous, hematolog- ic and hepatobiliary disorders and presence of isolat- ed congenital heart block (ICHB). Recent studies have also demonstrated the existence of a "lupus pattern" of dyslipoproteinemia characterized by very high lev- els of VLDL cholesterol and triglycerides (TG) and low HDL cholesterol levels. 1 This case approaches NLS lipid abnormalities not explained by use of drugs like corticosteroids, anticon- vulsants, antihypertensives, estrogen and/or proges- terone, known to impair lipid profile, 2 nor by condi- By Jonas Ribas, MD, MSc, Carla B. R. Ribas, MD, MSc, and Janaina S. Tirapelle, MD. Neonatal Lupus Associated with Dyslipidemia Neonatal lupus syndrome (NLS) is a rare disease characterized by an autoimmune process associated with the presence of auto antibodies in maternal fetal circulation against proteins SSA/Ro and SSB/La. Figs. 1 and 2. Erythematous, fine-scaling mask-like plaque on the face of a three-month-old male born to a clinically asymptomatic mother. Appearance of a periorbital ‘‘owl-eye’’ rash. September 2010 | Practical Dermatology | 17 Clinical Case Report
Transcript of Neonatal Lupus Associated with Dyslipidemia Case Report.pdfof dyslipoproteinemia characterized by...
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A male patient, three months old,born at term gestation without com-plications, was taken by his motherfor medical evaluation due to a two-month evolution, large, bilateral, ery-thematous, fine-scaling mask-likeplaque on the face, which was wors-ened by sun exposure (Figs. 1,2).After long evaluation, the possibilityof Neonatal Lupus Syndrome wassuspected and ANA of the patientwas collected. Serum sample was ini-tially rejected due to its unexpectedlyheavy hyperlipidemic nature, whichpersisted in subsequent samples.Hemogram and Lypidogram testswere collected, presenting the fol-lowing results: eight percent eosinophilia, 70 percentlymphocytosis, and triglyceride levels of 976mg/dL.ANA was ultimately tested and demonstrated posi-tive in 1:640. Skin biopsy confirmed the diagnosis ofLupus. Electrocardiogram has shown no impairment.Parallel mother evaluation demonstrated speckled-patterned ANA anti-Hep 1:2560 and anti-Ro 1:3200,despite lack of rheumatologic disease diagnosis andsymptoms.
No nephrologic impairment was revealed duringevaluation of the child. Skin lesions remitted afterfour months of treatment. Triglyceride levels reachednormality after eight months. The patient was subject-ed to evaluation every three months during his firstyear of age and semiannually until four years old andremained asymptomatic during monitoring.
DiscussionThe neonatal lupus syndrome (NLS) is a rare diseasecharacterized by an autoimmune process associatedwith the presence of auto antibodies in maternal fetalcirculation against proteins SSA/Ro and SSB/La. Itsclinical manifestations include cutaneous, hematolog-ic and hepatobiliary disorders and presence of isolat-ed congenital heart block (ICHB). Recent studies havealso demonstrated the existence of a "lupus pattern"of dyslipoproteinemia characterized by very high lev-els of VLDL cholesterol and triglycerides (TG) andlow HDL cholesterol levels.1
This case approaches NLS lipid abnormalities notexplained by use of drugs like corticosteroids, anticon-vulsants, antihypertensives, estrogen and/or proges-terone, known to impair lipid profile,2 nor by condi-
By Jonas Ribas, MD, MSc, Carla B. R. Ribas, MD, MSc, and Janaina S. Tirapelle, MD.
Neonatal Lupus Associated with Dyslipidemia Neonatal lupus syndrome (NLS) is a rare disease characterized by an autoimmune process associatedwith the presence of auto antibodies in maternal fetal circulation against proteins SSA/Ro and SSB/La.
Figs. 1 and 2. Erythematous, fine-scaling mask-like plaque on the face of a three-month-old maleborn to a clinically asymptomatic mother. Appearance of a periorbital ‘‘owl-eye’’ rash.
September 2010 | Practical Dermatology | 17
Clinical Case Report
