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Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation, but...
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Rheumatic Heart Disease I: Introduction01:23

Rheumatic Heart Disease I: Introduction

Rheumatic heart disease or RHD is a chronic condition that results from rheumatic fever, causing permanent damage to the heart valves.Etiology and Risk FactorsIt primarily arises from rheumatic fever, an inflammatory disease that can develop after untreated or inadequately treated group A streptococcal (GAS) pharyngitis. Streptococcus spreads through direct contact with oral or respiratory secretions. While the bacteria are the causative agents, factors like malnutrition, overcrowding, poor...
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...
Inflammatory Bowel Disease III: Crohn's Disease01:25

Inflammatory Bowel Disease III: Crohn's Disease

Crohn’s disease is a chronic, relapsing form of inflammatory bowel disease characterized by segmental, transmural inflammation that can affect any part of the gastrointestinal tract. Its pathogenesis arises from a combination of genetic susceptibility, environmental exposures, epithelial barrier dysfunction, and immune dysregulation. Together, these factors lead to an exaggerated immune response against components of the gut microbiome.Genetic and Environmental InfluencesMultiple genetic...
Cerebral Edema ll: Pathophysiology01:22

Cerebral Edema ll: Pathophysiology

Vasogenic edema is a major form of cerebral edema characterized by abnormal accumulation of fluid in the brain’s extracellular space due to disruption of the blood–brain barrier (BBB). The BBB is a specialized structure composed of endothelial cells connected by tight junctions, supported by astrocytic endfeet and a basement membrane. Under normal conditions, it tightly regulates the movement of ions, proteins, and solutes between the bloodstream and brain parenchyma. When this barrier loses...

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Related Experiment Video

Updated: May 20, 2026

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Recurrent vasculitis in cystic fibrosis.

Lareina Wujanto1, Clare Ross

  • 1Dermatology Department, Ealing Hospital, Southall, Middlesex, UK. reinawujanto@gmail.com

BMJ Case Reports
|July 14, 2012
PubMed
Summary

Cystic fibrosis (CF) can cause rare skin issues like CF-related vasculitis, appearing as a purpuric rash. This vasculitis, an extrapulmonary manifestation, can occur during infective exacerbations of CF.

Area of Science:

  • Medicine
  • Dermatology
  • Pulmonology

Background:

  • Cystic Fibrosis (CF) is a genetic disorder affecting multiple organs.
  • Extrapulmonary manifestations of CF are less common but significant.
  • Vasculitis is an inflammation of blood vessels.

Observation:

  • A 22-year-old male with known Cystic Fibrosis (CF) presented with a recurrent palpable purpuric rash.
  • The rash, identified as CF-related vasculitis, predominantly affected the lower limbs.
  • The onset coincided with an infective exacerbation of CF (IECF), but also occurred independently.

Findings:

  • CF-related vasculitis is a rare, extrapulmonary manifestation of Cystic Fibrosis.
  • The condition presents as a purpuric rash, typically on the lower extremities.

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  • The rash showed spontaneous remission in some instances but required immunosuppression in others.
  • Implications:

    • Highlights the diverse clinical spectrum of Cystic Fibrosis.
    • Suggests a need for heightened awareness of vasculitis in CF patients.
    • Underscores the importance of managing both pulmonary and extrapulmonary complications in CF.