Goodpasture's syndrome (GS) is characterized by lung hemorrhage and glomerulonephritis and caused by autoimmune reaction between anti-glomerular basement membrane (GBM) autoantibodies and the alpha 3 (type IV) collagen chain. Some reports suggested that patients with anti-GBM autoantibody could be related with other autoimmune diseases including Graves' disease. We report a case of 14-year-old girl with Graves' disease treated with PTU for 4 years, who was admitted because of hemoptysis and dyspnea. Laboratory values included a serum creatinine value of 0.7 mg/dL, BUN 22 mg/dL, hemoglobin 3.9 g/mm3 and albumin 3.2 mg/dL. The thyroid function tests showed normal serum T3 and free T4, suppressed TSH, and elevated thyroglobulin Ab and TSH-R-Ab levels. Urinalysis showed 2+ for protein and many dysmorphic RBC/HPF. Both anti-GBM Ab and pANCA were positive serologically. In renal biopsy, the glomeruli showed mesangial proliferation and crescent formation with linear deposits of IgG along the GBM. This case is to represent the GS of patient with Graves' disease referring to medical documents.
Adolescent ; Anti-Glomerular Basement Membrane Disease* ; Autoantibodies ; Autoimmune Diseases ; Basement Membrane ; Biopsy ; Collagen ; Creatinine ; Dyspnea ; Female ; Glomerulonephritis ; Graves Disease* ; Hemoptysis ; Hemorrhage ; Humans ; Immunoglobulin G ; Lung ; Methylprednisolone ; Thyroglobulin ; Thyroid Function Tests ; Urinalysis

Lateral eyebrow mass with primary skull lesion are rare in pediatric population. Although epidermoid cyst and dermoid cyst are the most commonly encountered skull lesions in pediatric population, Langerhans cell histiocytosis (LCH) is rarely reported. We report a case of LCH arising from the lateral eyebrow with osteolytic lesion involving the frontal bone. A 5-year-old boy was presented with a hard, fixed mass in his lateral eyebrow. Contrast magnetic resonance imaging revealed inhomogeneous enhancement of the mass with direct invasion of the frontal bone and adjacent dura mater. Under general anesthesia, linear incision at the lateral eyebrow region was made. Intraoperative evaluation revealed hard, fixed and well-defined soft tissue mass. The final extirpated mass was 2.5 x 2.4 cm in size, and was accompanied by a 1 x 1 cm sized defect on the frontal bone with intact dura mater. The surgical wound was closed primarily by a layer-by-layer fashion. Histologic examination was later performed for definite diagnosis. The histologic examination revealed abnormal proliferation of Langerhans cell with granuloma formation. Radionuclide bone scan and positron emission tomography was taken and revealed free of multi-organ involvement. At 3 months after surgery, natural looking contour at the lateral eyebrow region was observed with no tumor recurrence. Differential diagnosis of the hard and fixed mass at the lateral eyebrow region affecting the primary skull lesion from pediatric population includes epidermoid cyst, dermoid cyst and LCH. Generally, brief physical examination with plain X-ray view can be performed for clinical evaluation, but for a definite diagnosis, contrast MRI may be helpful.
Anesthesia, General ; Child ; Dermoid Cyst ; Diagnosis, Differential ; Dura Mater ; Epidermal Cyst ; Eyebrows ; Frontal Bone ; Granuloma ; Histiocytosis ; Histiocytosis, Langerhans-Cell ; Humans ; Magnetic Resonance Imaging ; Physical Examination ; Positron-Emission Tomography ; Recurrence ; Skull

Fusarium species are common soil saprophytes and plant pathogens. In humans, several species have been recognized as agents of superficial infections. Disseminated Fusariosis have been increasingly described in immunocompromised patients, especially in neutropenic patients. The prognosis is very poor despites antifungal therapy. This is the report of Fusarium oxysporum infection in a 6-year-old patient with relapsed acute leukemia and prolonged neutropenia. The patient presented with persistent fever and multiple erythematous papules with central necrosis or vesicle. Fuasrium oxysporum was isolated and cultured from a skin biopsy specimen. Initially, the patient failed to respond to conventional amphotericin B but recovered after treatment was switched to liposomal amphotericin B and voriconazole.
Amphotericin B* ; Biopsy ; Child* ; Drug Therapy* ; Fever ; Fusariosis* ; Fusarium ; Humans ; Immunocompromised Host ; Leukemia ; Necrosis ; Neutropenia ; Plants ; Prognosis ; Skin ; Soil

No abstract available.
Foot* ; Hand* ; Tendons*

No abstract available.

Neonatal lupus erythematosus(NLE) is a rare disease characterized by the transplacental passage from the mother to the fetus of autoantibodies, particularly autoantibodies of Ro family. The patient with NLE exhibits one or more of the following findings: congenital heart block, cutaneous lupus lesions, hepatobiliary disease and hematologic disorders(thrombocytopenia, anemia). We report a case of NLE in a 2-week-old male infant, born of a clinically asymptomatic mother, presenting multiple, round, target-like lesions which have not been reported in the English and Korean literature. Both infant and mother were positive for anti-SSA/Ro and anti-SSB/La.
Autoantibodies ; Fetus ; Heart Block ; Humans ; Infant ; Male ; Mothers ; Rare Diseases ; Skin*