Related Experiment Video

Updated: Jul 19, 2026

Three-Dimensional Cell Culture Models to Investigate the Epithelial Barrier in Eosinophilic Esophagitis
03:23

Three-Dimensional Cell Culture Models to Investigate the Epithelial Barrier in Eosinophilic Esophagitis

Published on: May 10, 2024

Faces of eosinophilic fasciitis in childhood

Ana I Quintero-Del-Rio1, Marilyn Punaro, Virginia Pascual

  • 1Department of Pediatrics, Division of Rheumatology, Texas Scottish Rite Hospital for Children and The University of Texas Southwestern Medical Center at Dallas, Dallas, Texas, USA. ana-quintero@omrf.ouhsc.edu

Journal of Clinical Rheumatology : Practical Reports on Rheumatic & Musculoskeletal Diseases
|October 17, 2006
PubMed

Insights

Eosinophilic fasciitis in children can present with unusual symptoms like Raynaud's phenomenon and hepatosplenomegaly. Early diagnosis is key, as this rare connective tissue disease often responds well to prednisone treatment.

Area of Science:

  • Pediatric Rheumatology
  • Connective Tissue Diseases
  • Rare Diseases

Background:

  • Eosinophilic fasciitis (EF) is a rare pediatric connective tissue disease.
  • Characterized by skin/soft tissue thickening, peripheral eosinophilia, elevated ESR, and hypergammaglobulinemia.
  • Clinical presentations and therapeutic responses in pediatric EF can vary.

Purpose of the Study:

  • To report three pediatric cases of eosinophilic fasciitis with atypical initial presentations.
  • To highlight variations in clinical manifestations and treatment responses compared to existing literature.
  • To emphasize the importance of differentiating EF from scleroderma in pediatric patients.

Main Methods:

  • Case series reporting on three pediatric patients diagnosed with eosinophilic fasciitis.
  • Clinical evaluation including physical examination, laboratory tests (aldolase, ESR), and muscle biopsy.
  • Assessment of therapeutic response to prednisone.

Main Results:

  • All three patients were female, with two presenting with Raynaud's phenomenon and one with hepatosplenomegaly.
  • Muscle involvement with weakness and elevated aldolase was noted in two patients.
  • All patients showed peripheral eosinophilia and responded favorably to prednisone therapy.

Conclusions:

  • Raynaud's phenomenon and hepatosplenomegaly can be part of the clinical spectrum of childhood eosinophilic fasciitis.
  • Distinguishing EF from scleroderma is crucial, especially when hand involvement and Raynaud's phenomenon are present.
  • Prompt identification and steroid-responsive treatment are vital for managing pediatric eosinophilic fasciitis.

Related Concept Videos

Rocky Mountain Spotted Fever01:26

Rocky Mountain Spotted Fever

Rocky Mountain Spotted Fever (RMSF) is a severe tick-borne illness caused by Rickettsia rickettsii, a Gram-negative, coccobacillary bacterium. This pathogen is an obligate intracellular parasite, requiring a host cell for replication. Transmission occurs through the bite of an infected tick. In the United States, the most important vectors are Dermacentor variabilis (American dog tick) and Dermacentor andersoni (Rocky Mountain wood tick), though other tick species may also serve as vectors.
Asthma I: Introduction01:28

Asthma I: Introduction

Asthma is a chronic inflammatory disorder of the airways characterized by variable airflow obstruction and heightened bronchial responsiveness to a wide range of triggers. The underlying inflammation leads to airway swelling, mucus hypersecretion, and smooth muscle constriction, all of which narrow the airway lumen and impede airflow. Clinically, asthma presents with recurrent episodes of wheezing, shortness of breath, chest tightness, and coughing, symptoms that typically vary in intensity and...
Amebiasis01:28

Amebiasis

Entamoeba histolytica, a protozoan parasite, is responsible for intestinal and extraintestinal amebiasis. Though a significant proportion of infections remain asymptomatic, approximately 50 million individuals annually are estimated to present with clinical disease, resulting in up to 100,000 deaths globally. The disease burden is disproportionately high in regions with lower socioeconomic status, such as parts of India, Africa, Mexico, and Latin America.Etiology and TransmissionThe infective...
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
Acne Infection01:27

Acne Infection

Acne is a multifactorial skin condition primarily affecting adolescents and young adults, with a global prevalence estimated to exceed 75% in this demographic. The condition is characterized by the formation of comedones (blackheads and whiteheads), papules, pustules, nodules, and, in severe cases, cysts, particularly in areas rich in sebaceous glands such as the face, neck, chest, and back. The pathogenesis involves increased sebum production, follicular hyperkeratinization, colonization by...
Asthma III: Clinical Manifestations01:13

Asthma III: Clinical Manifestations

Asthma presents with a characteristic pattern of episodic respiratory symptoms that reflect underlying airway inflammation, bronchoconstriction, and mucus hypersecretion. Although severity varies among individuals, certain clinical manifestations are considered hallmarks of the disorder and often guide diagnosis and assessment.Respiratory SymptomsA persistent cough is one of the most common early features of asthma. It is frequently dry and tends to worsen at night or in the early morning,...