ABSTRACT
Kimura’s disease is a rare condition and typically presents as non-tender subcutaneous swellings in the head and neck region, usually in the pre-auricular and submandibular areas. It is associated with lymphadenopathy (both local and distal), marked peripheral eosinophilia, and an elevated IgE level. It can easily be mistaken for a malignant disorder. Fine needle aspiration can be misleading, and a diagnosis is established only by histopathological examination. Renal involvement, which may affect up to 60% of patients, is the only systemic manifestation. We report a case of Kimura’s disease in a Malay patient who was associated with steroid-responsive nephrotic syndrome.
ABSTRACT
Anti-nuclear antibody (ANA) negative systemic lupus erythematosus (SLE) occurs in about 4-13% of SLE cases. A small group of ANA negative SLE patients with positive anti-Ro antibodies usually present with typical vasculitic skin lesions which can be associated with photosensitivity, renal disease, congenital heart block or neonatal lupus. We present a case of a persistently ANA negative patient who presented with joint pain, rashes, mouth ulcer and alopecia. Clinical diagnosis of systemic lupus erythematosus was made even though ANA was negative. She was started on steroids and went into remission. Later, she developed several episodes of convulsions associated with fever and prominent vasculitic lesions. The patient was also found to have microscopic hematuria, proteinuria, anemia and thrombocytopenia. Renal biopsy showed lupus nephritis class 1B. Due to the prominent skin lesions, we performed anti-extractable nuclear antigens (ENA) antibodies test and anti-Ro turned out to be positive. The final diagnosis was ANA negative SLE (Ro lupus) with cutaneous, renal, musculoskeletal, hematological and cerebral Involvement.