Bullous systemic lupus erythematosus and membranous lupus nephritis in a Filipino child

@#<p style="text-align: justify;">Bullous eruptions are rare cutaneous manifestations of systemic lupus erythematosus. We report a case of an 8-year old Filipino girl with vesiculobullous systemic lupus erythematosus (SLE) and membranous lupus nephritis on kidney biopsy who presented with clinical nephrotic features of generalized edema, proteinuria, hypoalbuminemia and hyperlipidemia. The 2012 Systemic Lupus International Collaborating Clinics (SLICC) classification criteria for SLE were met. Immunohistopathologic examination of the skin lesion revealed a sub-epidermal split with neutrophilic infiltrates along the dermo-epidermal junction, moderate perivascular, periadnexal and interstitial infiltrates composed of predominantly neutrophils with neutrophilic dusts, lymphocytes, plasma cells, rare eosinophils and increased dermal mucin. Direct immunofluorescence showed strong continuous linear IgG deposits along the basement membrane and weak linear IgM and IgA deposition along the basement membrane zone (BMZ). To our knowledge, this is the first report of vesiculobullous SLE in a Filipino child. This case is a rare form of cutaneous lupus in children. Bullous SLE (BSLE) should be considered in the differential diagnosis of children presenting with generalized bullous eruptions.</p>

Lupus Membranous Nephropathy with Atypical Serology / 대한신장학회잡지

Glomerulonephritis is the common manifestation of SLE and occurs in approximately 60-75% of patients. However, renal involvement as an initial presentation of SLE is not frequent finding. Serum complement levels and anti-ds DNA titer are useful tests in assessing the activity of lupus nephritis, but the level of anti-ds DNA is often normal in patients with pure membranous disease. With the above characteristics, this case shows atypical serology that is positive anti-La/SSB which is related with the absence of nephritis.