Article Text

GRANULOMATOUS DISEASE IN CHILDHOOD CONFIRMING A RARE DIAGNOSIS
  1. S M Lima1,
  2. E Costa1,
  3. A Oliveira1,
  4. M Costa1,
  5. V Monteiro1,
  6. C Godinho1,
  7. F Rocha2
  1. 1Department of Paediatrics, Hospital Sao Miguel, Oliveira Azemeis, Portugal
  2. 2Department of Pulmonology, Hospital Sao Miguel, Oliveira Azemeis, Portugal

Abstract

Introduction Granulomatous diseases are rare in children and, when they occur, differential diagnosis with other clinical entities with different treatment and prognosis is required. Sarcoidosis is a systemic granulomatous disease of unknown etiology, relatively rare in childhood. Children younger than 4 years present with the triad of skin, joint, and eye involvement without typical pulmonary disease in older children, involvement of the lungs, lymph nodes, and eyes predominates.

Case report An 8yearold Caucasian girl presented with erythema nodosum associated with fatigue, malaise and low grade fever for four days. Pulmonary Xray revealed bilateral mediastinal lymphadenopathies, with biopsy of these lymph nodes showing noncaseating epithelioid cell granulomas. Bronchoalveolar lavage fluid revealed lymphocytosis with an augmented CD4CD8 Tlymphocyte ratio. Lung functional tests showed a combined restrictive and obstructive pattern. Tuberculosis was excluded. The diagnosis of sarcoidosis was made stage I and treatment was started with prednisolone, which was maintained for six months, with good response.

After a threeyear follow up, she presents with no abnormalities on chest Xray, normal functional lung tests and no relapses.

Comments The authors present this case to alert for a rare disease in the paediatric population that should be suspected in the presence of typical clinical findings. Differential diagnosis with other clinical entities must be made and an extensive workup with invasive procedures and a multidisciplinary approach is needed. Prolonged and close followup is necessary to monitor treatment and possible relapses. The main goal of therapeutic intervention, when indicated, is to prevent fibrosis.

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.