Lymphangioma circumscriptum is a benign hamartomatous malformation involving the lymphatic system of skin and subcutaneous tissue. It can involve any part of the body with maximum predilection for proximal parts of limbs. Vulvar involvement is quite infrequent. Based on the aetiopathogenesis, it can be either primary or secondary, with the secondary form being relatively more common in vulva. We report an exceedingly rare case of primary lymphangioma circumscriptum in a post-menopausal female presenting with multiple warty, papulo-nodular lesions on both labia majora. The patient was diagnosed histopathologically and managed surgically by wide local excision with primary closure.
Epstein-Barr virus-associated lymphocytic cholangitis in a child with X-linked lymphoproliferative syndrome Dear Editor, X-linked lymphoproliferative disease (XLP) is a primary immunodeficiency disease caused by a defect in signalling lymphocyte-activation molecule (SLAM)-associated protein encoded by SH2 domain-containing 1A (SH2D1A) gene. This results in an increased susceptibility to Epstein-Barr virus (EBV)-related infections and lymphoproliferation. 1 Lymphocytic cholangitis (LC) is a rare porto-hepatic disease usually identified on histopathology by predominant infiltration of lymphocytes and plasma cells in portal tracts. 2 LC is recognized as a distinct entity in veterinary medicine especially in feline species, and however, reports in humans are scarce. 2,3 To the best of our knowledge, LC has not been reported in children. A 4-year-old boy presented with one-month history of high-grade intermittent fever and diffuse dull-aching abdominal pain. Examination revealed pallor, generalized lymphadenopathy, progressively increasing liver size (span: 15 cm) and conjugated hyperbilirubinemia over next 2 weeks. Splenomegaly was not noted. On investigations, he had anaemia, leukocytosis with lymphocytic predominance, conjugated hyperbilirubinemia and elevated liver enzymes (Table 1). Bone marrow biopsy showed reactive changes. Fine-needle aspiration cytology of cervical lymph node showed reactive lymphoid hyperplasia. Common bacterial and viral infectious causes were ruled out. Work-up for Wilson disease and autoimmune diseases including autoimmune hepatitis work-up were negative (Table 1). Epstein-Barr virus (EBV) deoxyribonucleic acid polymerase chain reaction (PCR) and EBV viral capsid antigen (VCA) IgG and IgM were positive whereas EBV nuclear antigen-1 IgG was negative suggesting an acute EBV infection. Immunoglobulin (Ig) levels (IgG, IgA, IgM) were normal. (Table 1). Urine examination was normal. Ultrasonography of abdomen suggested hepatomegaly with increased echogenicity and normal portal structures. Echocardiography was normal. Bacterial and fungal cultures of CSF were sterile. Contrast-enhanced computed tomography (CECT) scan of brain was normal. Positron emission tomography (PET) scan did not show any significant fluorodeoxyglucose (FDG) uptake in any organ. He was treated with intravenous ceftriaxone for 2 weeks for meningitis and oral valganciclovir (350 mg/d) for 3 weeks for suppression of EBV replication. 4
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