Systemic lupus erythematosus and granulomatous lymphadenopathy.

BACKGROUND: Systemic lupus erythematosus (SLE) is known to present with a wide variety of clinical manifestations. Lymphadenopathy is frequently observed in children with SLE and may occasionally be the presenting feature. SLE presenting with granulomatous changes in lymph node biopsy is rare. These features may also cause diagnostic confusion with other causes of granulomatous lymphadenopathy. CASE PRESENTATION: We report 12 year-old female who presented with generalized lymphadenopathy associated with intermittent fever as well as weight loss for three years. She also had developed anasarca two years prior to presentation. On presentation, she had growth failure and delayed puberty. Lymph node biopsy revealed granulomatous features. She developed a malar rash, arthritis and positive ANA antibodies over the course of next two months and showed WHO class II lupus nephritis on renal biopsy, which confirmed the final diagnosis of SLE. She was started on oral prednisolone and hydroxychloroquine with which her clinical condition improved, and she is currently much better under regular follow up. CONCLUSION: Generalized lymphadenopathy may be the presenting feature of SLE and it may preceed the other symptoms of SLE by many years as illustrated by this patient. Granulomatous changes may rarely be seen in lupus lymphadenitis. Although uncommon, in children who present with generalized lymphadenopathy along with prolonged fever and constitutional symptoms, non-infectious causes like SLE should also be considered as a diagnostic possibility.

Atypical presentation of scleroderma in infancy.

We present a case of a female child who had developed progressive skin tightening of whole body, sclerodactyly, and severe contracture of all joints with restricted opening of mouth with disease onset at 6 weeks of life. The child also had anemia, failure to thrive, recurrent diarrhea, and ascites. Skin biopsy revealed characteristic histopathological features suggestive of scleroderma. Echocardiography revealed thrombus in the right atrium extending to inferior vena cava. Serological markers (antinuclear antibody, antiscl 70, anti-double-stranded DNA, anticentromere antibody) were negative. To our knowledge, this case of scleroderma with disease onset at 6 weeks of life could possibly be among youngest cases reported in the literature. Also, thrombosis in inferior vena cava in a child with scleroderma is being reported for the first time.

Chromoblastomycosis in Nepal: a study of 13 cases.

BACKGROUND: Chromoblastomycosis is a chronic fungal infection caused by several pigmented fungi commonly seen in tropical and subtropical climates. AIM: To evaluate the epidemiologic, clinical and pathological characteristics of chromoblastomycosis in our patients. METHODS: This retrospective and prospective study was conducted at the Manipal Teaching Hospital, Pokhara, Nepal. Clinical features and histopathology of all the cases diagnosed as chromoblastomycosis during the last eight years were studied. RESULTS: A total of 13 cases of chromoblastomycosis were diagnosed during the period of 1999-2006. The disease was seen predominantly in middle-aged male farmers and those from rural areas. The lesions commonly involved the lower extremity and were single or multiple in number. They clinically presented as verrucous or nodular growths. Out of these 13 cases, three were diagnosed clinically as squamous cell carcinoma and one as psoriasis. The histopathological features included sclerotic bodies in 12 cases (92%), microabscess formation in 10 cases (76.9%), pseudoepitheliomatous hyperplasia in nine cases (69.2%) and granuloma in eight cases (61.5%). CONCLUSION: Farming is the commonest occupation in patients with chromoblastomycosis. Early histological diagnosis helps in effective management of the condition.