Pericardial effusion as a crucial presentation of Erdheim-Chester disease in a hemodialysis patient: an overlooked diagnosis.

[erdheim-chester disease]

Erdheim-Chester disease (ECD ) is a rare non-Langerhans cell histiocytosis whose hallmark is tissue infiltration by CD 68 -positive , CD 1 a-negative and usually S-100 protein-positive foamy non-Langerhans histiocytes and mononuclear cells . Here , we report a hemodialysis (HD ) patient who presented with fever and pericardial effusion . We performed pericardiocentesis with pericardial biopsy and the histological findings indicated ECD . We administered intravenous methylprednisolone pulse therapy (250 mg /d ) followed by oral prednisolone (50 mg /d ). The patient 's fever gradually subsided and there was no recurrence of pericardial effusion . This is the first report of an HD patient with ECD . We suggest that ECD be considered in the differential diagnosis of new HD patients who present with pericardial effusion , especially when this did not improve following increased dose of HD .

Diseases
Validation

Diseases presenting "fever" symptom

22q11.2 deletion syndrome

acute rheumatic fever

alexander disease

allergic bronchopulmonary aspergillosis

canavan disease

carcinoma of the gallbladder

child syndrome

congenital toxoplasmosis

cushing syndrome

cystinuria

dracunculiasis

erdheim-chester disease

esophageal adenocarcinoma

esophageal carcinoma

familial mediterranean fever

focal myositis

hodgkin lymphoma, classical

lamellar ichthyosis

legionellosis

locked-in syndrome

malignant atrophic papulosis

neonatal adrenoleukodystrophy

neuralgic amyotrophy

oculocutaneous albinism

papillon-lefèvre syndrome

pyomyositis

pyruvate dehydrogenase deficiency

scrub typhus

severe combined immunodeficiency

sneddon syndrome

systemic capillary leak syndrome

triple a syndrome

typhoid

waldenström macroglobulinemia

wolf-hirschhorn syndrome

This symptom has already been validated