Background We and others have noticed consistent staining of sebaceous glands with PReferentially expressed Antigen in MElanoma (PRAME). We aimed to determine whether PRAME was as sensitive, specific, and interpretable as adipophilin for distinguishing sebaceous neoplasms (SNs) from other neoplasms. Methods Twenty SNs and 32 control cases were stained for PRAME and adipophilin. Extent of staining was scored as follows: 0, no staining; 1, <5% positivity; 2, 5% to 50% positivity; and 3, >50% positivity. Intensity was scored as negative, weak, moderate, or strong. A composite score was determined by adding the scores for extent and intensity. Results PRAME had positive composite scores in all 20 SNs in the more differentiated areas, whereas adipophilin had positive composite scores in 19/20 cases. PRAME showed positivity in the basaloid cells in 15/16 cases, whereas adipophilin was positive in 14. Among controls, PRAME and adipophilin had positive composite scores in 3/32 cases and 6/32 cases, respectively. Conclusions PRAME and adipophilin are comparable in terms of distribution and intensity for staining sebocytes. In the basaloid cells, PRAME expression is often more diffuse and easier to detect than adipophilin. In comparing the SNs to the controls, PRAME was more sensitive and more specific than adipophilin. PRAME could be used as an additional marker of sebaceous differentiation in everyday practice.
A 2-year-old male presented to our emergency department with lethargy, increased work of breathing, and abdominal pain of 1-day duration. Two weeks prior to presentation, his primary care physician clinically diagnosed him with pneumonia when he presented with high fever, runny nose, and eye drainage. No chest radiograph was done at this time. There were also sick family members with similar symptoms. He received 5 days of azithromycin.He took longer to recover than the rest of his family, but was close to normal activity in 10 days. Four days prior to admission, he seemed to relapse when his mother noticed his chest "caving in" and she could "see his heart beating hard and fast." He also had decreased oral intake and no urine output during the 12 hours prior to admission. During the day of admission, his legs began to swell, and he appeared to have swelling throughout his body. There had been no associated fever, nausea, vomiting, diarrhea, or rash.He had no significant past medical history. Examination on admission revealed substernal and subcostal retractions and use of accessory abdominal muscles for breathing. On auscultation of his heart he had a gallop. Also noted were hepatomegaly of 5 cm below the right costal margin and bilateral edema of the lower extremities.Initial laboratory evaluation included normal electrolytes except for an anion gap of 2.16 mmol/L (normal, 8-16 mmol/L). Lactate was 1.43 mmol/L (normal, 0.36-1.25 mmol/L), and aspartate transaminase was 55 U/L (normal, 10-40 U/L). Chest radiograph showed marked cardiomegaly and bilateral diffuse interstitial and parenchymal attenuation consistent with pulmonary edema. A cardiac echo was then performed, which demonstrated a severely dilated left ventricle with a Z-score of +6.2, severely decreased systolic function with shortening fraction of <12% (ejection fraction of <30%), and an otherwise structurally normal heart. Cardiac laboratory values revealed a BNP (B-type natriuretic peptide) of 2638 pg/mL (normal, 0-99) and a troponin of 0.194 ng/mL (0-0.026). He was diagnosed with a dilated cardiomyopathy presumably secondary to viral myocarditis. Diagnosis Myocarditis Hospital CourseHe was admitted to the pediatric intensive care unit for further workup and management where he was started on milrinone at 0.5 μg/kg/min and lasix intravenous 7 mg/kg every 6 hours. A peripherally inserted central catheter line and a radial arterial line were placed, and after obtaining blood to evaluate possible etiologies of his cardiac disease, intravenous immunoglobulin 2 g over 24 hrs ×1 was given for the treatment of myocarditis.Laboratory evaluation for the etiology of his myocarditis included a respiratory panel, which was only positive for rhinovirus, and antibodies for cytomegalovirus, Epstein-Barr virus, mycoplasma, human herpesvirus-6, and parvovirus B19, all of which were negative. Blood cultures were also negative.With the above interventions, there was improvement in his pulse pressure, metabolic acidosis, and clinical exam, with decreased work of breathi...
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